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Information on EC 7.1.1.2 - NADH:ubiquinone reductase (H+-translocating) and Organism(s) Homo sapiens and UniProt Accession Q9BRQ8
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EC Tree
7.1.1.2 NADH:ubiquinone reductase (H+-translocating)IUBMB Comments
The enzyme is a very large complex that participates in electron transfer chains of mitochondria and aerobic bacteria, transferring electrons from NADH to the ubiquinone pool. Reversed electron transport through this enzyme can reduce NAD+ to NADH.
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- 1-methyl-4-phenylpyridinium
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photoluminescence
The taxonomic range for the selected organisms is: Homo sapiens
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria, Archaea
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria, Archaea
Synonyms
complex i, complex 1, nadh dehydrogenase, mitochondrial complex i, grim-19, respiratory complex i, respiratory chain complex i, nadh:ubiquinone oxidoreductase, mitochondrial nadh dehydrogenase, ndh-1, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
13 kDa differentiation-associated protein
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-
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CDA016
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Cell adhesion protein SQM1
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-
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Cell death-regulatory protein GRIM-19
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-
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CGI-39
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-
-
-
CI-11KD
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-
-
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CI-12KD
-
-
-
-
CI-14.8KD
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-
-
-
CI-14KD
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-
-
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CI-15 kDa
-
-
-
-
CI-16KD
-
-
-
-
CI-17.3KD
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-
-
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CI-17.8KD
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-
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CI-18 kDa
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-
-
-
CI-18Kd
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-
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CI-19.3KD
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-
-
-
CI-19KD
-
-
-
-
CI-20KD
-
-
-
-
CI-21KD
-
-
-
-
CI-22.5Kd
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-
-
-
CI-23KD
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-
-
-
CI-27KD
-
-
-
-
CI-28.5KD
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-
-
-
CI-29.9KD
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-
-
-
CI-29KD
-
-
-
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CI-30KD
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-
-
-
CI-31KD
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-
-
-
CI-38.5KD
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-
-
-
CI-39KD
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-
-
-
CI-40KD
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-
-
-
CI-42.5KD
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-
-
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CI-42KD
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-
-
-
CI-49KD
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-
-
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CI-51KD
-
-
-
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CI-75KD
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-
-
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CI-78KD
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-
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CI-9.5
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-
-
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CI-9KD
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-
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CI-AGGG
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-
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CI-AQDQ
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-
-
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CI-ASHI
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-
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CI-B12
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-
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CI-B14
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-
-
-
CI-B14.5a
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CI-B14.5b
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CI-B15
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CI-B16.6
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CI-B17
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CI-B17.2
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-
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CI-B18
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-
-
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CI-B22
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-
-
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CI-B8
-
-
-
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CI-B9
-
-
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CI-KFYI
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-
-
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CI-MLRQ
-
-
-
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CI-MNLL
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-
-
-
CI-MWFE
-
-
-
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CI-PDSW
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-
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CI-PGIV
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CI-SGDH
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-
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CIB17.2
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-
-
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coenzyme Q reductase
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-
-
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complex I
-
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complex I (electron transport chain)
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-
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complex I (mitochondrial electron transport)
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-
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complex I (NADH:Q1 oxidoreductase)
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complex I dehydrogenase
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Complex I-11KD
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Complex I-12KD
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Complex I-14.8KD
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-
-
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Complex I-14KD
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Complex I-15 kDa
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Complex I-16KD
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-
-
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Complex I-17.3KD
-
-
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-
Complex I-17.8KD
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Complex I-18 kDa
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-
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Complex I-18Kd
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-
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Complex I-19.3KD
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-
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Complex I-19KD
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Complex I-20KD
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-
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Complex I-21KD
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Complex I-22.5Kd
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-
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Complex I-23KD
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Complex I-27KD
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-
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Complex I-28.5KD
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Complex I-29.9KD
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Complex I-29KD
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-
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Complex I-30KD
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-
-
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Complex I-38.5KD
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-
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Complex I-39KD
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-
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Complex I-40KD
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-
-
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Complex I-42.5KD
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-
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Complex I-42KD
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-
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Complex I-49KD
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-
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Complex I-51KD
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-
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Complex I-75KD
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-
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Complex I-78KD
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-
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Complex I-9.5KD
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-
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Complex I-9KD
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-
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Complex I-AGGG
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-
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Complex I-AQDQ
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-
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Complex I-ASHI
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Complex I-B12
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Complex I-B14
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Complex I-B14.5a
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Complex I-B14.5b
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Complex I-B15
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Complex I-B16.6
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Complex I-B17
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Complex I-B17.2
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Complex I-B18
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Complex I-B22
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Complex I-B8
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Complex I-B9
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Complex I-KFYI
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Complex I-MLRQ
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-
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Complex I-MNLL
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Complex I-MWFE
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-
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Complex I-PDSW
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-
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Complex I-PGIV
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Complex I-SGDH
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dihydronicotinamide adenine dinucleotide-coenzyme Q reductase
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DPNH-coenzyme Q reductase
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DPNH-ubiquinone reductase
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electron transfer complex I
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Gene associated with retinoic-interferon-induced mortality 19 protein
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GGHPW
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GRIM-19
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Hypothetical protein Walter
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Internal NADH dehydrogenase
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mitochondrial electron transport complex 1
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mitochondrial electron transport complex I
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NADH coenzyme Q1 reductase
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NADH-coenzyme Q oxidoreductase
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NADH-coenzyme Q reductase
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NADH-CoQ oxidoreductase
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NADH-CoQ reductase
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NADH-ferricyanide reductase
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NADH-Q6 oxidoreductase
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NADH-quinone reductase
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NADH-ubiquinone oxidoreductase
NADH-ubiquinone reductase
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NADH-ubiquinone-1 reductase
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NADH:Q oxidoreductase
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NADH:ubiquinone oxidoreductase complex
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Protein P1
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reduced nicotinamide adenine dinucleotide-coenzyme Q reductase
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reductase, ubiquinone
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type I dehydrogenase
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ubiquinone reductase
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Ubiquinone-binding protein
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NADH-ubiquinone oxidoreductase
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
redox reaction
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oxidation
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reduction
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-
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PATHWAY SOURCE
PATHWAYS
BRENDA
-
-, -, -, -, -, -
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SYSTEMATIC NAME
IUBMB Comments
NADH:ubiquinone oxidoreductase
The enzyme is a very large complex that participates in electron transfer chains of mitochondria and aerobic bacteria, transferring electrons from NADH to the ubiquinone pool. Reversed electron transport through this enzyme can reduce NAD+ to NADH.
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CAS REGISTRY NUMBER
COMMENTARY
9028-04-0
-
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
NADH + oxidized coenzyme Q1 + 5 H+[side1]
NAD+ + reduced coenzyme Q1 + 4 H+[side2]
-
-
-
?
NADH + H+ + ubiquinone
NAD+ + ubiquinol
-
NADH:ubiquinone oxidoreductase or complex I is a large multisubunit assembly of the mitochondrial inner membrane that channels high-energy electrons from metabolic NADH into the electron transport chain. Its dysfunction is associated with a range of progressive neurological disorders, often characterized by a very early onset and short devastating course. Reduction in cellular complex I activity leads to a depolarization of the mitochondrial membrane potential, resulting in a decreased supply of mitochondrial ATP to the Ca2+-ATPases of the intracellular stores and thus to a reduced Ca2+ content of these stores
-
-
?
NADH + oxidized coenzyme Q1 + 5 H+[side1]
NAD+ + reduced coenzyme Q1 + 4 H+[side2]
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-
-
?
additional information
?
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-
NADH:ubiquinone oxidoreductase (EC 1.6.5.3) constitutes the entry point of electrons in the electron transport chain. Mitochondrial NADH:ubiquinone oxidoreductase or complex I (CI) is a frequently affected enzyme in cases of mitochondrial disorders
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-
?
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
NADH + H+ + ubiquinone
NAD+ + ubiquinol
-
NADH:ubiquinone oxidoreductase or complex I is a large multisubunit assembly of the mitochondrial inner membrane that channels high-energy electrons from metabolic NADH into the electron transport chain. Its dysfunction is associated with a range of progressive neurological disorders, often characterized by a very early onset and short devastating course. Reduction in cellular complex I activity leads to a depolarization of the mitochondrial membrane potential, resulting in a decreased supply of mitochondrial ATP to the Ca2+-ATPases of the intracellular stores and thus to a reduced Ca2+ content of these stores
-
-
?
additional information
?
-
-
NADH:ubiquinone oxidoreductase (EC 1.6.5.3) constitutes the entry point of electrons in the electron transport chain. Mitochondrial NADH:ubiquinone oxidoreductase or complex I (CI) is a frequently affected enzyme in cases of mitochondrial disorders
-
-
?
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
1-(4-(4-nitrophenyl)piperazin-1-yl)-4-(thiophen-2-yl)butan-1-one
-
compound inhibits NADH:ubiquinone oxidoreductase, does stimulate glucose uptake in vitro
-
2',3'-dideoxycytidine
-
0.001 mM prevents the phosphorylation of the NDUFB11 subunit of complex I
3'-azido-3'-deoxythymidine
-
0.01 and 0.05 mM prevent the phosphorylation of the NDUFB11 subunit of complex I. This is associated with a decrease in complex I activity
3'-azido-3'-deoxythymidine monophosphate
-
0.15 mM prevents the phosphorylation of the NDUFB11 subunit of complex I. This is associated with a decrease in complex I activity
3-(thiophen-2-ylthio)-1-(4-(4-(trifluoromethyl)phenyl)piperazin-1-yl)propan-1-one
-
compound effectively stimulates glucose uptake in vitro and inhibits NADH:ubiquinone oxidoreductase
-
4-(thiophen-2-yl)-1-(4-(4-(trifluoromethyl)phenyl)piperazin-1-yl)butan-1-one
-
compound effectively stimulates glucose uptake in vitro 2.57fold and inhibits NADH:ubiquinone oxidoreductase. Compound will not provoke adverse drug-drug interactions, yet be readily metabolised, avoid rapid excretion, with a short half-life, and have good tissue distribution
-
4-(thiophen-2-yl)-1-(4-(4-(trifluoromethyl)phenyl)piperidin-1-yl)butan-1-one
-
compound effectively stimulates glucose uptake in vitro and inhibits NADH:ubiquinone oxidoreductase
-
5-phenyl-1-(4-(4-(trifluoromethyl)phenyl)piperazin-1-yl)pentan-1-one
-
compound effectively stimulates glucose uptake in vitro and inhibits NADH:ubiquinone oxidoreductase
-
additional information
-
COX I mutant cybrids show a 80% reduction in complex I enzymatic activity in isolated mitochondria as compared with control cybrids
-
rotenone
-
inhibits complex I followed by apoptosis. 40% decrease of dopamine content suppresses rotenone-induced apoptosis. 30% increase of dopamine content by inhibition of dopamine metabolism enhances rotenone-induced apoptosis. Depletion of intracellular dopamine using reserpine (0.0001-0.01 mM) also prevents rotenone-induced apoptosis, and this effect is counteracted by dopamine (0.01-0.1 mM) replenishment. Inhibition of dopamine reverse transport increases cytosolic dopamine and enhances rotenone-induced apoptosis. Rotenone induces dopamine redistribution from vesicles to the cytosol. Rotenone stimulates reactive oxygen species and protein carbonylation and decreases the antioxidant glutathione. Addition of the antioxidant N-acetylcysteine (3 mM), prevents dopamine being expelled from vesicles and inhibits rotenone-induced apoptosis
rotenone
-
RCC-I inactivation causes a dramatic decrease in mitochondrial NO that is not affected by Mn (III) porphyrin 5,10,15,20-tetrakis(benzoic acid) porphyrin. RCC-I inactivation drastically increases mitochondrial reactive oxygen species, which is prevented when mitochondrial nitric oxide synthase is inhibited or cells are treated with Mn (III) porphyrin 5,10,15,20-tetrakis(benzoic acid) porphyrin. Inactivation of RCC-I in SHSY cells dramatically increases tyrosine nitration of mitochondrial proteins that is fully prevented when mitochondrial nitric oxide synthase is inactivated. RCC-I inactivation leads cytochrome c to diffuse into the cytoplasm indicating its release from the mitochondria
rotenone
-
reduces complex I activity, which is accompanied by increasing production of cellular superoxide
rotenone
-
when complex I activity is chronically reduced by 80% using 100 nanomol rotenone, the percentage of moving mitochondria decreases from 79% to 50% and reduce their velocity by 30%
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
rotenone
-
chronic rotenone treatment (100 nanomol, 72 h) increases fully assembled complex I in human skin fibroblasts
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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
1-acylglycerophosphocholine o-acyltransferase deficiency
Cardiolipin remodeling by ALCAT1 links oxidative stress and mitochondrial dysfunction to obesity.
3-hydroxyacyl-coa dehydrogenase deficiency
Mitochondrial respiratory chain complex I deficiency with clinical and biochemical features of long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.
Abnormalities, Multiple
Cell-Based Blood Biomarkers for Myalgic Encephalomyelitis/Chronic Fatigue Syndrome.
Abortion, Spontaneous
A single nucleotide polymorphism in the MTOR gene is associated with recurrent spontaneous abortion in the Chinese female population.
Abortion, Spontaneous
Expression of GRIM-19 in unexplained recurrent spontaneous abortion and possible pathogenesis.
Abruptio Placentae
Abruptio placentae risk and genetic variations in mitochondrial biogenesis and oxidative phosphorylation: replication of a candidate gene association study.
Acidosis
Acidosis Drives the Reprogramming of Fatty Acid Metabolism in Cancer Cells through Changes in Mitochondrial and Histone Acetylation.
Acidosis
Deficiency of the reduced nicotinamide adenine dinucleotide dehydrogenase component of complex I of mitochondrial electron transport. Fatal infantile lactic acidosis and hypermetabolism with skeletal-cardiac myopathy and encephalopathy.
Acidosis
Effects of acidosis and ATP depletion on cardiac muscle electron transfer complex I.
Acidosis
Mild Acidosis Protects Neurons during Oxygen-Glucose Deprivation by Reducing Loss of Mitochondrial Respiration.
Acidosis
Respiratory chain defect of myocardial mitochondria in idiopathic dilated cardiomyopathy of Doberman pinscher dogs.
Acidosis
Severe multisystem pathology, metabolic acidosis, mitochondrial dysfunction, and early death associated with an X-linked AIFM1 variant.
Acidosis
[Metabolic study in a child with Leigh's syndrome and deficient activity in complex I of the respiratory chain]
Acidosis, Lactic
A case of mitochondrial myopathy, lactic acidosis and complex I deficiency.
Acidosis, Lactic
A new disease-related mutation for mitochondrial encephalopathy lactic acidosis and strokelike episodes (MELAS) syndrome affects the ND4 subunit of the respiratory complex I.
Acidosis, Lactic
A novel mutation in the human complex I NDUFS7 subunit associated with Leigh syndrome.
Acidosis, Lactic
An autopsy case of mitochondrial encephalomyopathy with prominent degeneration in olivo-ponto-cerebellar system.
Acidosis, Lactic
An unusual patient with the neonatal Marfan phenotype and mitochondrial complex I deficiency.
Acidosis, Lactic
Aplasia of the retinal vessels combined with optic nerve hypoplasia, neonatal epileptic seizures, and lactic acidosis due to mitochondrial complex I deficiency.
Acidosis, Lactic
Bilateral infantile cataractogenesis in a patient with deficiency of complex I, a mitochondrial electron transport chain enzyme.
Acidosis, Lactic
Clinical presentation of mitochondrial respiratory chain defects in NADH-coenzyme Q reductase and cytochrome oxidase: clues to pathogenesis of Leigh disease.
Acidosis, Lactic
Complex I (reduced nicotinamide-adenine dinucleotide-coenzyme Q reductase) deficiency in two patients with probable Leigh syndrome.
Acidosis, Lactic
Congenital deficiency of two polypeptide subunits of the iron-protein fragment of mitochondrial complex I.
Acidosis, Lactic
Congenital lactic acidosis, cerebral cysts and pulmonary hypertension in an infant with FOXRED1 related complex 1 deficiency.
Acidosis, Lactic
Congenital lactic acidosis, cerebral cysts and pulmonary hypertension in an infant with FOXRED1 related complex I deficiency.
Acidosis, Lactic
Deep Sequencing Reveals Novel Genetic Variants in Children with Acute Liver Failure and Tissue Evidence of Impaired Energy Metabolism.
Acidosis, Lactic
Defective mitochondrial translation caused by a ribosomal protein (MRPS16) mutation.
Acidosis, Lactic
Deficiency of the iron-sulfur clusters of mitochondrial reduced nicotinamide-adenine dinucleotide-ubiquinone oxidoreductase (complex I) in an infant with congenital lactic acidosis.
Acidosis, Lactic
Deficiency of the reduced nicotinamide adenine dinucleotide dehydrogenase component of complex I of mitochondrial electron transport. Fatal infantile lactic acidosis and hypermetabolism with skeletal-cardiac myopathy and encephalopathy.
Acidosis, Lactic
Development and characterization of polyspecific anti-mitochondrion antibodies for proteomics studies on in toto tissue homogenates.
Acidosis, Lactic
Discovery of AdipoRon analogues as novel AMPK activators without inhibiting mitochondrial complex I.
Acidosis, Lactic
Disease-causing mitochondrial heteroplasmy segregated within induced pluripotent stem cell clones derived from a patient with MELAS.
Acidosis, Lactic
Disproportionate deficiency of iron-sulfur clusters and subunits of complex I in mitochondrial encephalomyopathy.
Acidosis, Lactic
Diverse phenotype in patients with complex I deficiency due to mutations in NDUFB11.
Acidosis, Lactic
Evidence of a wide spectrum of cardiac involvement due to ACAD9 mutations: Report on nine patients.
Acidosis, Lactic
Familial cardiomyopathy with cataracts and lactic acidosis: a defect in complex I (NADH-dehydrogenase) of the mitochondria respiratory chain.
Acidosis, Lactic
Fatal mitochondrial myopathy, lactic acidosis, and complex I deficiency associated with a heteroplasmic A --> G mutation at position 3251 in the mitochondrial tRNALeu(UUR) gne.
Acidosis, Lactic
Fatal neonatal lactic acidosis with respiratory insufficiency due to complex I and IV deficiency.
Acidosis, Lactic
Functional and morphological abnormalities of mitochondria harbouring the tRNA(Leu)(UUR) mutation in mitochondrial DNA derived from patients with maternally inherited diabetes and deafness (MIDD) and progressive kidney disease.
Acidosis, Lactic
Immunohistochemical analysis of the oxidative phosphorylation complexes in skeletal muscle from patients with mitochondrial DNA encoded tRNA gene defects.
Acidosis, Lactic
Introduction of an additional pathway for lactate oxidation in the treatment of lactic acidosis and mitochondrial dysfunction in Caenorhabditis elegans.
Acidosis, Lactic
Is the mitochondrial complex I ND5 gene a hot-spot for MELAS causing mutations?
Acidosis, Lactic
Lactic Acidosis Complicating Metformin and Non-Nucleoside Reverse Transcriptase Inhibitor Combination Therapy: A Smoldering Threat in the Post-HAART Era.
Acidosis, Lactic
LHON/MELAS overlap mutation in ND1 subunit of mitochondrial complex I affects ubiquinone binding as revealed by modeling in Escherichia coli NDH-1.
Acidosis, Lactic
Lifetime exercise intolerance with lactic acidosis as key manifestation of novel compound heterozygous ACAD9 mutations causing complex I deficiency.
Acidosis, Lactic
Mitochondrial complex I deficiency caused by a deleterious NDUFA11 mutation.
Acidosis, Lactic
Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase.
Acidosis, Lactic
Mitochondrial complex I mutations in Caenorhabditis elegans produce cytochrome c oxidase deficiency, oxidative stress and vitamin-responsive lactic acidosis.
Acidosis, Lactic
Mitochondrial DNA mutations in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS).
Acidosis, Lactic
Mitochondrial Encephalopathy and Optic Neuropathy Due to m.10158 MT-ND3 Complex I Mutation Presenting in an Adult Patient: Case Report and Review of the Literature.
Acidosis, Lactic
Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes syndrome and NADH-CoQ reductase deficiency.
Acidosis, Lactic
Mitochondrial variations in the MT-ND4 and MT-TL1 genes are associated with male infertility.
Acidosis, Lactic
Mutated NDUFS6 is the cause of fatal neonatal lactic acidemia in Caucasus Jews.
Acidosis, Lactic
Mutation of mitochondrial DNA G13513A presenting with Leigh syndrome, Wolff-Parkinson-White syndrome and cardiomyopathy.
Acidosis, Lactic
Mutations in mitochondrially encoded complex I enzyme as the second common cause in a cohort of Chinese patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes.
Acidosis, Lactic
Mutations in the accessory subunit NDUFB10 result in isolated complex I deficiency and illustrate the critical role of intermembrane space import for complex I holoenzyme assembly.
Acidosis, Lactic
Neonatal lactic acidosis, complex I/IV deficiency, and fetal cerebral disruption.
Acidosis, Lactic
Neonatal multiorgan failure due to ACAD9 mutation and complex I deficiency with mitochondrial hyperplasia in liver, cardiac myocytes, skeletal muscle, and renal tubules.
Acidosis, Lactic
Novel mitochondrial DNA ND5 mutation in a patient with clinical features of MELAS and MERRF.
Acidosis, Lactic
Nuclear DNA origin of mitochondrial complex I deficiency in fatal infantile lactic acidosis evidenced by transnuclear complementation of cultured fibroblasts.
Acidosis, Lactic
Predominant cerebellar volume loss as a neuroradiologic feature of pediatric respiratory chain defects.
Acidosis, Lactic
Respiratory chain complex I deficiency: an underdiagnosed energy generation disorder.
Acidosis, Lactic
Riboflavin enhances the assembly of mitochondrial cytochrome c oxidase in C. elegans NADH-ubiquinone oxidoreductase mutants.
Acidosis, Lactic
Role of organic cation/carnitine transporter 1 in uptake of phenformin and inhibitory effect on complex I respiration in mitochondria.
Acidosis, Lactic
Sequence analysis of nuclear genes encoding functionally important complex I subunits in children with encephalomyopathy.
Acidosis, Lactic
Severe complex I deficiency in a case of neonatal-onset lactic acidosis and fatal liver failure.
Acidosis, Lactic
Severe congenital lactic acidosis and hypertrophic cardiomyopathy caused by an intronic variant in NDUFB7.
Acidosis, Lactic
Sporadic Intragenic Inversion of the Mitochondrial DNA MTND1 Gene Causing Fatal Infantile Lactic Acidosis.
Acidosis, Lactic
Succinate and the shortcut to the cure of metformin-induced lactic acidosis.
Acidosis, Lactic
The addition of ketone bodies alleviates mitochondrial dysfunction by restoring complex I assembly in a MELAS cellular model.
Acidosis, Lactic
The m.3890G>A/MT-ND1 mtDNA rare pathogenic variant: Expanding clinical and MRI phenotypes.
Acidosis, Lactic
The MELAS mutations 3946 and 3949 perturb the critical structure in a conserved loop of the ND1 subunit of mitochondrial complex I.
Acidosis, Lactic
The mitochondrial 13513G > A mutation is most frequent in Leigh syndrome combined with reduced complex I activity, optic atrophy and/or Wolff-Parkinson-White.
Acidosis, Lactic
The role of complex I genes in MELAS: a novel heteroplasmic mutation 3380G>A in ND1 of mtDNA.
Acidosis, Lactic
Tissue specific defect of complex I of the mitochondrial respiratory chain.
Acidosis, Lactic
Vitamin-responsive complex I deficiency in a myopathic patient with increased activity of the terminal respiratory chain and lactic acidosis.
Acidosis, Lactic
[Complex I (NADH coenzyme-Q-reductase) deficiency, MELAS syndrome and hypertrophic cardiomyopathy]
Acne Vulgaris
Diet in Acne: Further Evidence for the Role of Nutrient Signalling in Acne Pathogenesis.
Acne Vulgaris
Evaluation of serum FoxO1, mTORC1, IGF-1, IGFBP-3 levels, and metabolic syndrome components in patients with acne vulgaris: A prospective case-control study.
Acquired Immunodeficiency Syndrome
Cellular rescue-assay aids verification of causative DNA-variants in mitochondrial complex I deficiency.
Acquired Immunodeficiency Syndrome
mTOR activity in AIDS-related diffuse large B-cell lymphoma.
Acquired Immunodeficiency Syndrome
Pancreatitis associated with human immunodeficiency virus infection: a matched case-control study.
Acquired Immunodeficiency Syndrome
Peliosis hepatis and sinusoidal dilation during infection by the human immunodeficiency virus (HIV). An ultrastructural study.
Acquired Immunodeficiency Syndrome
Relationships Between Adipose Mitochondrial Function, Serum Adiponectin, and Insulin Resistance in Persons With HIV After 96 Weeks of Antiretroviral Therapy.
Acute Kidney Injury
Inhibition of Mitochondrial Complex I Aggravates Folic Acid-Induced Acute Kidney Injury.
Acute Kidney Injury
Rotenone Protects Against Acetaminophen-Induced Kidney Injury by Attenuating Oxidative Stress and Inflammation.
Acute Kidney Injury
TSC1 deletion in fibroblasts alleviates lipopolysaccharide-induced acute kidney injury.
Acute Lung Injury
Lung transcriptome of a COVID-19 patient and systems biology predictions suggest impaired surfactant production which may be druggable by surfactant therapy.
Acute Lung Injury
Mitochondrial respiratory complex I regulates neutrophil activation and severity of lung injury.
Acute Lung Injury
Participation of mTOR Complex 1 in TLR2 and TLR4 Induced Neutrophil Activation and Acute Lung Injury.
Adenocarcinoma
A new Cu(II)-O-Carvacrotinate complex: Synthesis, characterization and biological activity.
Adenocarcinoma
A novel binuclear hydrazone-based Cd(II) complex is a strong pro-apoptotic inducer with significant activity against 2D and 3D pancreatic cancer stem cells.
Adenocarcinoma
A photoactivatable Ru (II) complex bearing 2,9-diphenyl-1,10-phenanthroline: A potent chemotherapeutic drug inducing apoptosis in triple negative human breast adenocarcinoma cells.
Adenocarcinoma
Antibody-mediated neutralization of soluble MIC significantly enhances CTLA4 blockade therapy.
Adenocarcinoma
Half-Sandwich Ru(p-cymene) Compounds with Diphosphanes: In Vitro and In Vivo Evaluation As Potential Anticancer Metallodrugs.
Adenocarcinoma
Investigation of in vitro anticancer and DNA strap interactions in live cells using carboplatin type Cu(II) and Zn(II) metalloinsertors.
Adenocarcinoma
K-Ras mutation-mediated IGF-1-induced feedback ERK activation contributes to the rapalog resistance in pancreatic ductal adenocarcinomas.
Adenocarcinoma
miRNA-1290 Promotes Aggressiveness in Pancreatic Ductal Adenocarcinoma by Targeting IKK1.
Adenocarcinoma
mTORC1 and mTORC2 Converge on the Arp2/3 Complex to Promote KrasG12D-Induced Acinar-to-Ductal Metaplasia and Early Pancreatic Carcinogenesis.
Adenocarcinoma
Single amino-acid changes that confer constitutive activation of mTOR are discovered in human cancer.
Adenocarcinoma
Synthesis, Spectral Characterization, DNA/ Protein Binding, DNA Cleavage, Cytotoxicity, Antioxidative and Molecular Docking Studies of Cu(II)Complexes Containing Schiff Base-bpy/Phen Ligands.
Adenocarcinoma
Synthesis, Structure, Properties And Biological Behaviour Of The Complex [Ru (H(2)L) Cl(2)].2H(2)O (H(4)L= 1,2-Cyclohexanediamminetetraacetic Acid).
Adenocarcinoma
Treatment of Pancreatic Cancer Patient-Derived Xenograft Panel with Metabolic Inhibitors Reveals Efficacy of Phenformin.
Adenocarcinoma of Lung
A new Cu(II)-O-Carvacrotinate complex: Synthesis, characterization and biological activity.
Adenocarcinoma of Lung
Azide-mediated unusual in situ transformation of Mannich base to Schiff-Mannich base and isolation of their Cu(II) complexes: crystal structure, theoretical inspection and anticancer activities.
Adenocarcinoma of Lung
Gene associated with retinoid-interferon-induced mortality-19 suppresses growth of lung adenocarcinoma tumor in vitro and in vivo.
Adenocarcinoma of Lung
MYG1 promotes proliferation and inhibits autophagy in lung adenocarcinoma cells via the AMPK/mTOR complex 1 signaling pathway.
Adenocarcinoma of Lung
Nonsense and missense mutation of mitochondrial ND6 gene promotes cell migration and invasion in human lung adenocarcinoma.
Adenocarcinoma of Lung
Targeting Human Lung Adenocarcinoma with a Suppressor of Mitochondrial Superoxide Production.
Adenoma
Mitochondrial DNA somatic mutations (point mutations and large deletions) and mitochondrial DNA variants in human thyroid pathology: a study with emphasis on Hürthle cell tumors.
Adenoma
Stromal Liver Kinase B1 [STK11] Signaling Loss Induces Oviductal Adenomas and Endometrial Cancer by Activating Mammalian Target of Rapamycin Complex 1.
Adenoma, Oxyphilic
Clonal expansion of mutated mitochondrial DNA is associated with tumor formation and complex I deficiency in the benign renal oncocytoma.
Adenoma, Oxyphilic
Decreased Mitochondrial DNA Content Drives OXPHOS Dysregulation in Chromophobe Renal Cell Carcinoma.
Adenoma, Oxyphilic
Early loss of mitochondrial complex I and rewiring of glutathione metabolism in renal oncocytoma.
Adenoma, Oxyphilic
Enhancement of mitochondrial biogenesis and paradoxical inhibition of lactate dehydrogenase mediated by 14-3-3? in oncocytomas.
Adenoma, Oxyphilic
Inducing cancer indolence by targeting mitochondrial Complex I is potentiated by blocking macrophage-mediated adaptive responses.
Adenoma, Oxyphilic
Loss of complex I due to mitochondrial DNA mutations in renal oncocytoma.
Adenoma, Oxyphilic
Mitochondrial DNA mutations distinguish bilateral multifocal renal oncocytomas from familial Birt-Hogg-Dubé tumors.
Adenoma, Oxyphilic
Renal oncocytoma characterized by the defective complex I of the respiratory chain boosts the synthesis of the ROS scavenger glutathione.
Adenoma, Oxyphilic
Respiratory chain complex I is a mitochondrial tumor suppressor of oncocytic tumors.
Adenoma, Oxyphilic
Respiratory complex I dysfunction due to mitochondrial DNA mutations shifts the voltage threshold for opening of the permeability transition pore toward resting levels.
Adenoma, Oxyphilic
Somatic and germline mutation in GRIM-19, a dual function gene involved in mitochondrial metabolism and cell death, is linked to mitochondrion-rich (Hurthle cell) tumours of the thyroid.
Adenomatous Polyposis Coli
Familial non-medullary thyroid carcinoma: clinico-pathological features, current knowledge and novelty regarding genetic risk factors.
Adenomatous Polyposis Coli
JNK Signaling Promotes Intestinal Tumorigenesis Through Activation of mTOR Complex 1 in Apc?716 Mice.
Adenomatous Polyposis Coli
Oncogenic mutations in Adenomatous Polyposis Coli (Apc) activate mechanistic Target of Rapamycin Complex 1 (mTORC1).
Adenomyosis
Interaction of M2 macrophages and endometrial cells induces downregulation of GRIM-19 in endometria of adenomyosis.
Adrenoleukodystrophy
The use of targeted genomic capture and massively parallel sequencing in diagnosis of Chinese Leukoencephalopathies.
Agenesis of Corpus Callosum
Defective mitochondrial translation caused by a ribosomal protein (MRPS16) mutation.
AIDS-Related Complex
Pancreatitis associated with human immunodeficiency virus infection: a matched case-control study.
AIDS-Related Complex
Peliosis hepatis and sinusoidal dilation during infection by the human immunodeficiency virus (HIV). An ultrastructural study.
Albuminuria
Deficiency in Mitochondrial Complex I Activity Due to Ndufs6 Gene Trap Insertion Induces Renal Disease.
Alcohol-Related Disorders
Role for mammalian target of rapamycin complex 1 signaling in neuroadaptations underlying alcohol-related disorders.
Alexander Disease
The use of targeted genomic capture and massively parallel sequencing in diagnosis of Chinese Leukoencephalopathies.
Alopecia
Folliculin, the product of the Birt-Hogg-Dube tumor suppressor gene, interacts with the adherens junction protein p0071 to regulate cell-cell adhesion.
Alzheimer Disease
Activities of mitochondrial respiratory chain complexes in platelets of patients with Alzheimer's disease and depressive disorder.
Alzheimer Disease
AMPK activation does not enhance autophagy in neurons in contrast to MTORC1 inhibition: different impact on ?-amyloid clearance.
Alzheimer Disease
Ca(2+)-dependent permeability transition and complex I activity in lymphoblast mitochondria from normal individuals and patients with Huntington's or Alzheimer's disease.
Alzheimer Disease
Differential Activation of mTOR Complex 1 Signaling in Human Brain with Mild to Severe Alzheimer's Disease.
Alzheimer Disease
Electroacupuncture ameliorates beta-amyloid pathology and cognitive impairment in Alzheimer disease via a novel mechanism involving activation of TFEB (transcription factor EB).
Alzheimer Disease
Elevated oxidative stress and decreased antioxidant function in the human hippocampus and frontal cortex with increasing age: implications for neurodegeneration in Alzheimer's disease.
Alzheimer Disease
Gene expression of ND4, a subunit of complex I of oxidative phosphorylation in mitochondria, is decreased in temporal cortex of brains of Alzheimer's disease patients.
Alzheimer Disease
Geographical Distribution and Diversity of Gut Microbial NADH:Ubiquinone Oxidoreductase Sequence Associated with Alzheimer's Disease.
Alzheimer Disease
Identification of genes associated with the effect of inflammation on the neurotransmission of vascular smooth muscle cell.
Alzheimer Disease
Isolated cerebral and cerebellar mitochondria produce free radicals when exposed to elevated CA2+ and Na+: implications for neurodegeneration.
Alzheimer Disease
Mitochondrial complex I abnormalities is associated with tau and clinical symptoms in mild Alzheimer's disease.
Alzheimer Disease
Mitochondrial variations in the MT-ND4 and MT-TL1 genes are associated with male infertility.
Alzheimer Disease
Modulation of mitochondrial complex I activity averts cognitive decline in multiple animal models of familial Alzheimer's Disease.
Alzheimer Disease
mTor is a signaling hub in cell survival: a mass spectrometry-based proteomics investigation.
Alzheimer Disease
mTORC2 (Rictor) in Alzheimer's Disease and Reversal of Amyloid-? Expression-Induced Insulin Resistance and Toxicity in Rat Primary Cortical Neurons.
Alzheimer Disease
Multivariate meta-analyses of mitochondrial complex I and IV in major depressive disorder, bipolar disorder, schizophrenia, Alzheimer disease, and Parkinson disease.
Alzheimer Disease
No association of mutations at nucleotide 5460 of mitochondrial NADH dehydrogenase with Alzheimer's disease.
Alzheimer Disease
Partial inhibition of mitochondrial complex I ameliorates Alzheimer's disease pathology and cognition in APP/PS1 female mice.
Alzheimer Disease
PSEN2 (presenilin 2) mutants linked to familial Alzheimer disease impair autophagy by altering Ca2+ homeostasis.
Alzheimer Disease
Reduced levels of mitochondrial complex I subunit NDUFB8 and linked complex I + III oxidoreductase activity in the TgCRND8 mouse model of Alzheimer's disease.
Alzheimer Disease
Structural understanding of 5-(4-hydroxy-phenyl)-N-(2-(5-methoxy-1H-indol-3-yl)-ethyl)-3-oxopentanamide as a neuroprotectant for Alzheimer's disease.
Alzheimer Disease
The expression of several mitochondrial and nuclear genes encoding the subunits of electron transport chain enzyme complexes, cytochrome c oxidase, and NADH dehydrogenase, in different brain regions in Alzheimer's disease.
Alzheimer Disease
The reduction of NADH ubiquinone oxidoreductase 24- and 75-kDa subunits in brains of patients with Down syndrome and Alzheimer's disease.
Amyotrophic Lateral Sclerosis
C9orf72 ALS-FTD: recent evidence for dysregulation of the autophagy-lysosome pathway at multiple levels.
Amyotrophic Lateral Sclerosis
C9orf72 and smcr8 mutant mice reveal MTORC1 activation due to impaired lysosomal degradation and exocytosis.
Amyotrophic Lateral Sclerosis
De novo single-nucleotide and copy number variation in discordant monozygotic twins reveals disease-related genes.
Amyotrophic Lateral Sclerosis
Major Histocompatibility Complex I Expression by Motor Neurons and Its Implication in Amyotrophic Lateral Sclerosis.
Amyotrophic Lateral Sclerosis
Mitochondrial complex I deficiency and ATP/ADP ratio in lymphocytes of amyotrophic lateral sclerosis patients.
Amyotrophic Lateral Sclerosis
Neurodegeneration induced by complex I inhibition in a cellular model of familial amyotrophic lateral sclerosis.
Amyotrophic Lateral Sclerosis
SETX (senataxin), the helicase mutated in AOA2 and ALS4, functions in autophagy regulation.
Anemia
Impact of Intravenous Iron on Oxidative Stress and Mitochondrial Function in Experimental Chronic Kidney Disease.
Anemia
Iron Deficiency without Anemia Decreases Physical Endurance and Mitochondrial Complex I Activity of Oxidative Skeletal Muscle in the Mouse.
Anemia
Iron deficiency without anemia is responsible for decreased left ventricular function and reduced mitochondrial complex I activity in a mouse model.
Anemia
Severe congenital lactic acidosis and hypertrophic cardiomyopathy caused by an intronic variant in NDUFB7.
Anemia, Hemolytic
Clinical Severity of PGK1 Deficiency Due To a Novel p.E120K Substitution Is Exacerbated by Co-inheritance of a Subclinical Translocation t(3;14)(q26.33;q12), Disrupting NUBPL Gene.
Anemia, Megaloblastic
A novel mutation in the thiamine responsive megaloblastic anaemia gene SLC19A2 in a patient with deficiency of respiratory chain complex I.
Anemia, Sideroblastic
A novel mutation in NDUFB11 unveils a new clinical phenotype associated with lactic acidosis and sideroblastic anemia.
Anemia, Sideroblastic
A recurring mutation in the respiratory complex 1 protein NDUFB11 is responsible for a novel form of X-linked sideroblastic anemia.
Anemia, Sideroblastic
Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease.
Anemia, Sideroblastic
Mitochondrial iron-sulfur cluster biogenesis from molecular understanding to clinical disease.
Angiofibroma
Clinical and Histologic Analysis of the Efficacy of Topical Rapamycin Therapy Against Hypomelanotic Macules in Tuberous Sclerosis Complex.
Angiomyolipoma
Human TSC-associated renal angiomyolipoma cells are hypersensitive to ER stress.
Anhedonia
Effects of omega-3 on behavioral and biochemical parameters in rats submitted to chronic mild stress.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Circulating nicotinamide adenine dinucleotide-ubiquinone oxidoreductase chain 6 is associated with disease activity of anti-neutrophil cytoplasmic antibody-associated vasculitis.
Antiphospholipid Syndrome
Activation of the Mechanistic Target of Rapamycin in SLE: Explosion of Evidence in the Last Five Years.
Antiphospholipid Syndrome
RapaLink-1 plays an antithrombotic role in antiphospholipid syndrome by improving autophagy both in vivo and vitro.
Aortic Coarctation
Sporadic Intragenic Inversion of the Mitochondrial DNA MTND1 Gene Causing Fatal Infantile Lactic Acidosis.
Aphasia
Patients with MELAS with negative myopathology for characteristic ragged-red fibers.
Apraxias
Congenital ocular motor apraxia associated with myopathy, external hydrocephalus and NADH dehydrogenase deficiency.
Apraxias
SETX (senataxin), the helicase mutated in AOA2 and ALS4, functions in autophagy regulation.
Arthritis
Survival patterns of Streptococcus suis serotypes 1 and 14 in porcine blood indicate cross-reactive bactericidal antibodies in naturally infected pigs.
Arthritis
[Ru(bpy)2(NO)SO3](PF6), a Nitric Oxide Donating Ruthenium Complex, Reduces Gout Arthritis in Mice.
Arthritis, Experimental
Gene associated with retinoid-interferon-induced mortality 19 attenuates murine autoimmune arthritis by regulation of th17 and treg cells.
Arthritis, Gouty
Simiao Decoction Alleviates Gouty Arthritis by Modulating Proinflammatory Cytokines and the Gut Ecosystem.
Arthritis, Rheumatoid
Activation of mechanistic target of rapamycin complex 1: the common link between rheumatoid arthritis and diabetes mellitus.
Arthritis, Rheumatoid
Gene associated with retinoid-interferon-induced mortality 19 attenuates murine autoimmune arthritis by regulation of th17 and treg cells.
Arthrogryposis
Mitochondrial complex I deficiency in a female with multiplex arthrogryposis congenita.
Arthrogryposis
Ragged-red fibers and complex I deficiency in a neonate with arthrogryposis congenita.
Asthenozoospermia
Downregulation of DJ-1 Fails to Protect Mitochondrial Complex I Subunit NDUFS3 in the Testes and Contributes to the Asthenozoospermia.
Asthma
Mitochondrial structural changes and dysfunction are associated with experimental allergic asthma.
Astrocytoma
Comparison of mitochondrial respiratory chain enzyme activities in rodent astrocytes and neurones and a human astrocytoma cell line.
Ataxia
A novel mutation in the mitochondrial tRNA(Pro) gene associated with late-onset ataxia, retinitis pigmentosa, deafness, leukoencephalopathy and complex I deficiency.
Ataxia
Early-onset cataracts, spastic paraparesis, and ataxia caused by a novel mitochondrial tRNAGlu (MT-TE) gene mutation causing severe complex I deficiency: a clinical, molecular, and neuropathologic study.
Ataxia
From ventriculomegaly to severe muscular atrophy: Expansion of the clinical spectrum related to mutations in AIFM1.
Ataxia
Infantile-onset spinocerebellar ataxia and mitochondrial recessive ataxia syndrome are associated with neuronal complex I defect and mtDNA depletion.
Ataxia
Investigating complex I deficiency in Purkinje cells and synapses in patients with mitochondrial disease.
Ataxia
Mitochondrial Complex 1 Activity Measured by Spectrophotometry Is Reduced across All Brain Regions in Ageing and More Specifically in Neurodegeneration.
Ataxia
Mitochondrial iron-sulfur cluster biogenesis from molecular understanding to clinical disease.
Ataxia
Mutations in AIFM1 cause an X-linked childhood cerebellar ataxia partially responsive to riboflavin.
Ataxia
NAD+ Regeneration Rescues Lifespan, but Not Ataxia, in a Mouse Model of Brain Mitochondrial Complex I Dysfunction.
Ataxia
SETX (senataxin), the helicase mutated in AOA2 and ALS4, functions in autophagy regulation.
Ataxia
Severe multisystem pathology, metabolic acidosis, mitochondrial dysfunction, and early death associated with an X-linked AIFM1 variant.
Ataxia
[Multiple symmetric lipomatosis associated to polyneuropathology, atrophy of the cerebellum and mitochondrial cytopathy]
Ataxia Telangiectasia
DNA-dependent protein kinase regulates lysosomal AMP-dependent protein kinase activation and autophagy.
Ataxia Telangiectasia
Neuroprotective effects of caffeine against complex I inhibition-induced apoptosis are mediated by inhibition of the Atm/p53/E2F-1 path in cerebellar granule neurons.
Atherosclerosis
Disruption of mTORC1 in Macrophages Decreases Chemokine Gene Expression and Atherosclerosis.
Atherosclerosis
Inhibition of mitochondrial activity ameliorates atherosclerosis in ApoE-/- mice via suppressing vascular smooth cell activation and macrophage foam cell formation.
Atherosclerosis
PRAS40 suppresses atherogenesis through inhibition of mTORC1-dependent pro-inflammatory signaling in endothelial cells.
Atherosclerosis
RapaLink-1 plays an antithrombotic role in antiphospholipid syndrome by improving autophagy both in vivo and vitro.
Atherosclerosis
[Association of mitochondrial genome mutations with lipofibrous plaques in human aortic intima].
Atrial Fibrillation
Pharmacogenetics of vitamin K antagonists and bleeding risk prediction in atrial fibrillation.
Atypical Squamous Cells of the Cervix
Decreased expression of GRIM-19 and its association with high-risk HPV infection in cervical squamous intraepithelial neoplasias and cancer.
Azotemia
Skeletal muscle mitochondrial depletion and dysfunction in chronic kidney disease.
Bacterial Infections
Evaluation of potential internal references for quantitative real-time RT-PCR normalization of gene expression in red drum (Sciaenops ocellatus).
Bacterial Infections
Function of GRIM-19, a Mitochondrial Respiratory Chain Complex I Protein, in Innate Immunity.
Bacterial Infections
GRIM-19: A Double-edged Sword that Regulates Anti-Tumor and Innate Immune Responses.
Barth Syndrome
Mitochondrial respiratory chain supercomplexes are destabilized in Barth Syndrome patients.
Bartonella Infections
The population structure of Lutzomyia verrucarum (Diptera: Psycodidae), a Bartonella bacilliformis and Leishmania peruviana vector in Peru.
Basal Ganglia Diseases
A2A adenosine receptor antagonists protect the striatum against rotenone-induced neurotoxicity.
Basal Ganglia Diseases
Transglutaminase 2 ablation leads to defective function of mitochondrial respiratory complex I affecting neuronal vulnerability in experimental models of extrapyramidal disorders.
Biliary Tract Neoplasms
The BMI1 inhibitor PTC-209 is a potential compound to halt cellular growth in biliary tract cancer cells.
Blindness
Differentiation-specific effects of LHON mutations introduced into neuronal NT2 cells.
Blindness
Functional analysis of lymphoblast and cybrid mitochondria containing the 3460, 11778, or 14484 Leber's hereditary optic neuropathy mitochondrial DNA mutation.
Blindness
Mutant NADH dehydrogenase subunit 4 gene delivery to mitochondria by targeting sequence-modified adeno-associated virus induces visual loss and optic atrophy in mice.
Blister
Down-regulation of mitochondrial acyl carrier protein in mammalian cells compromises protein lipoylation and respiratory complex I and results in cell death.
Bone Resorption
Mitochondrial complex I activity suppresses inflammation and enhances bone resorption by shifting macrophage-osteoclast polarization.
Bone Resorption
mTORC1 Activation in Osteoclasts Prevents Bone Loss in a Mouse Model of Osteoporosis.
Bone Resorption
TSC1 regulates osteoclast podosome organization and bone resorption through mTORC1 and Rac1/Cdc42.
Brain Damage, Chronic
Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage.
Brain Death
Combinatorial glucose, nicotinic acid and N-acetylcysteine therapy has synergistic effect in preclinical C. elegans and zebrafish models of mitochondrial complex I disease.
Brain Death
Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease.
Brain Diseases
A missense mutation in the mitochondrial ND5 gene associated with a Leigh-MELAS overlap syndrome.
Brain Diseases
A mitochondrial encephalomyopathy with cardiomyopathy. A case revealing a defect of complex I in the respiratory chain.
Brain Diseases
A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy.
Brain Diseases
A new disease-related mutation for mitochondrial encephalopathy lactic acidosis and strokelike episodes (MELAS) syndrome affects the ND4 subunit of the respiratory complex I.
Brain Diseases
A novel AIFM1 mutation expands the phenotype to an infantile motor neuron disease.
Brain Diseases
A novel mitochondrial ND5 (MTND5) gene mutation giving isolated exercise intolerance.
Brain Diseases
A novel mutation in the mitochondrial tRNA(Ser(AGY)) gene associated with mitochondrial myopathy, encephalopathy, and complex I deficiency.
Brain Diseases
A novel ND3 mitochondrial DNA mutation in three Korean children with basal ganglia lesions and complex I deficiency.
Brain Diseases
A novel NDUFV1 gene mutation in complex I deficiency in consanguineous siblings with brainstem lesions and Leigh syndrome.
Brain Diseases
A Single Intravenous Injection of AAV-PHP.B-hNDUFS4 Ameliorates the Phenotype of Ndufs4
-/-
Mice.
Brain Diseases
Complex I (reduced nicotinamide-adenine dinucleotide-coenzyme Q reductase) deficiency in two patients with probable Leigh syndrome.
Brain Diseases
Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome.
Brain Diseases
De novo mutations in the mitochondrial ND3 gene as a cause of infantile mitochondrial encephalopathy and complex I deficiency.
Brain Diseases
Deficiency of the reduced nicotinamide adenine dinucleotide dehydrogenase component of complex I of mitochondrial electron transport. Fatal infantile lactic acidosis and hypermetabolism with skeletal-cardiac myopathy and encephalopathy.
Brain Diseases
Deletion of the Complex I Subunit NDUFS4 Adversely Modulates Cellular Differentiation.
Brain Diseases
Disproportionate deficiency of iron-sulfur clusters and subunits of complex I in mitochondrial encephalomyopathy.
Brain Diseases
Dysfunction of mitochondrial respiratory chain complex I in neurological disorders: genetics and pathogenetic mechanisms.
Brain Diseases
Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome.
Brain Diseases
Immunohistochemical analysis of the oxidative phosphorylation complexes in skeletal muscle from patients with mitochondrial DNA encoded tRNA gene defects.
Brain Diseases
Impaired hypoxic pulmonary vasoconstriction in a mouse model of Leigh syndrome.
Brain Diseases
Insights into the pathogenic character of a common NUBPL branch-site mutation associated with mitochondrial disease and complex I deficiency using a yeast model.
Brain Diseases
LHON/MELAS overlap mutation in ND1 subunit of mitochondrial complex I affects ubiquinone binding as revealed by modeling in Escherichia coli NDH-1.
Brain Diseases
Liver-specific mitochondrial respiratory chain complex I deficiency in fatal influenza encephalopathy.
Brain Diseases
Metformin delays neurological symptom onset in a mouse model of neuronal complex I deficiency.
Brain Diseases
Mitochondrial DNA mutations in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS).
Brain Diseases
Mitochondrial encephalomyopathies in childhood. II. Clinical manifestations and syndromes.
Brain Diseases
Mitochondrial Encephalopathy and Optic Neuropathy Due to m.10158 MT-ND3 Complex I Mutation Presenting in an Adult Patient: Case Report and Review of the Literature.
Brain Diseases
Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes syndrome and NADH-CoQ reductase deficiency.
Brain Diseases
Mitochondrial network complexity and pathological decrease in complex I activity are tightly correlated in isolated human complex I deficiency.
Brain Diseases
Mitochondrial proteome analysis reveals depression of the Ndufs3 subunit and activity of complex I in diabetic rat brain.
Brain Diseases
Mitochondrial targeting of human NADH dehydrogenase (ubiquinone) flavoprotein 2 (NDUFV2) and its association with early-onset hypertrophic cardiomyopathy and encephalopathy.
Brain Diseases
Multiple defects of the respiratory chain including complex II in a family with myopathy and encephalopathy.
Brain Diseases
Mutant NDUFV2 subunit of mitochondrial complex I causes early onset hypertrophic cardiomyopathy and encephalopathy.
Brain Diseases
Mutation of mitochondrial DNA G13513A presenting with Leigh syndrome, Wolff-Parkinson-White syndrome and cardiomyopathy.
Brain Diseases
Mutations in mitochondrially encoded complex I enzyme as the second common cause in a cohort of Chinese patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes.
Brain Diseases
Mutations in ND subunits of complex I are an important genetic cause of childhood mitochondrial encephalopathies.
Brain Diseases
Necrotizing encephalopathy and macrocephaly with mitochondrial complex I deficiency.
Brain Diseases
New familial mitochondrial encephalopathy with macrocephaly, cardiomyopathy, and complex I deficiency.
Brain Diseases
Novel mutations of ND genes in complex I deficiency associated with mitochondrial encephalopathy.
Brain Diseases
Oxidative metabolites of 5-S-cysteinylnorepinephrine are irreversible inhibitors of mitochondrial complex I and the alpha-ketoglutarate dehydrogenase and pyruvate dehydrogenase complexes: possible implications for neurodegenerative brain disorders.
Brain Diseases
Pathogenetic mechanisms in hereditary dysfunctions of complex I of the respiratory chain in neurological diseases.
Brain Diseases
PI3K-mTOR-S6K Signaling Mediates Neuronal Viability via Collapsin Response Mediator Protein-2 Expression.
Brain Diseases
Progressive encephalopathy and complex I deficiency associated with mutations in MTND1.
Brain Diseases
Severe congenital lactic acidosis and hypertrophic cardiomyopathy caused by an intronic variant in NDUFB7.
Brain Diseases
Severe multisystem pathology, metabolic acidosis, mitochondrial dysfunction, and early death associated with an X-linked AIFM1 variant.
Brain Diseases
SLC25A10 biallelic mutations in intractable epileptic encephalopathy with complex I deficiency.
Brain Diseases
The m.3890G>A/MT-ND1 mtDNA rare pathogenic variant: Expanding clinical and MRI phenotypes.
Brain Diseases
The MELAS mutations 3946 and 3949 perturb the critical structure in a conserved loop of the ND1 subunit of mitochondrial complex I.
Brain Diseases
[Mitochondrial encephalopathy due to complex I deficiency. Brain tissue biopsy findings and clinical course following pharmacological]
Brain Injuries
Attenuation of oxidative damage by targeting mitochondrial complex I in neonatal hypoxic-ischemic brain injury.
Brain Injuries
Comparison of Effects of Metformin, Phenformin, and Inhibitors of Mitochondrial Complex I on Mitochondrial Permeability Transition and Ischemic Brain Injury.
Brain Injuries
Critical Role of Flavin and Glutathione in Complex I-Mediated Bioenergetic Failure in Brain Ischemia/Reperfusion Injury.
Brain Injuries
Mdivi-1 ameliorates early brain injury after subarachnoid hemorrhage via the suppression of inflammation-related blood-brain barrier disruption and endoplasmic reticulum stress-based apoptosis.
Brain Injuries
Recombinant human erythropoietin protects against brain injury through blunting the mTORC1 pathway in the developing brains of rats with seizures.
Brain Injuries
The oxygen free radicals originating from mitochondrial complex I contribute to oxidative brain injury following hypoxia-ischemia in neonatal mice.
Brain Injuries, Traumatic
Beneficial Effects of Early mTORC1 Inhibition after Traumatic Brain Injury.
Brain Injuries, Traumatic
Succinate and the shortcut to the cure of metformin-induced lactic acidosis.
Brain Ischemia
Reverse electron transfer results in a loss of flavin from mitochondrial complex I: Potential mechanism for brain ischemia reperfusion injury.
Brain Ischemia
The IFN-beta and retinoic acid-induced cell death regulator GRIM-19 is upregulated during focal cerebral ischemia.
Brain Neoplasms
Combined Modulation of Tumor Metabolism by Metformin and Diclofenac in Glioma.
Brain Neoplasms
Epigenetic regulation of survivin by Bmi1 is cell type specific during corticogenesis and in gliomas.
Brain Neoplasms
Inhibition of mitochondrial respiration prevents BRAF-mutant melanoma brain metastasis.
Brain Neoplasms
Mitochondrial 10398A>G NADH-Dehydrogenase Subunit 3 of Complex I Is Frequently Altered in Intra-Axial Brain Tumors in Malaysia.
Branchio-Oto-Renal Syndrome
The human B22 subunit of the NADH-ubiquinone oxidoreductase maps to the region of chromosome 8 involved in branchio-oto-renal syndrome.
Breast Diseases
Evaluating mitochondrial DNA in patients with breast cancer and benign breast disease.
Breast Neoplasms
A breast cancer stem cell-selective, mammospheres-potent osmium(VI) nitrido complex.
Breast Neoplasms
Activation of the Ir-N(pyridine) Bond in Half-Sandwich Tethered Iridium(III) Complexes.
Breast Neoplasms
An A10398G mitochondrial DNA alteration is related to increased risk of breast cancer, and associates with Her2 positive receptor.
Breast Neoplasms
Anticancer action of cubé insecticide: correlation for rotenoid constituents between inhibition of NADH:ubiquinone oxidoreductase and induced ornithine decarboxylase activities.
Breast Neoplasms
Association between mitochondrial genetic variation and breast cancer risk: The Multiethnic Cohort.
Breast Neoplasms
Biomarker signatures of mitochondrial NDUFS3 in invasive breast carcinoma.
Breast Neoplasms
cis-[PtCl2(4,7-H-5-methyl-7-oxo[1,2,4]triazolo[1,5-a]pyrimidine)2]: a sterically restrictive new cisplatin analogue. Reaction kinetics with model nucleobases, DNA interaction studies, antitumor activity, and structure-activity relationships.
Breast Neoplasms
Critical role of arachidonic acid-activated mTOR signaling in breast carcinogenesis and angiogenesis.
Breast Neoplasms
Crystal structure, optical properties, DFT analysis of new morpholine based Schiff base ligands and their copper(II) complexes: DNA, protein docking analyses, antibacterial study and anticancer evaluation.
Breast Neoplasms
Curcumin synergistically increases effects of ?-interferon and retinoic acid on breast cancer cells in vitro and in vivo by up-regulation of GRIM-19 through STAT3-dependent and STAT3-independent pathways.
Breast Neoplasms
Dichloroacetate enhances apoptotic cell death via oxidative damage and attenuates lactate production in metformin-treated breast cancer cells.
Breast Neoplasms
Down-regulation of GRIM-19 is associated with STAT3 overexpression in breast carcinomas.
Breast Neoplasms
Down-Regulation of NDUFB9 Promotes Breast Cancer Cell Proliferation, Metastasis by Mediating Mitochondrial Metabolism.
Breast Neoplasms
Effectiveness and molecular interactions of the clinically active mTORC1 inhibitor everolimus in combination with tamoxifen or letrozole in vitro and in vivo.
Breast Neoplasms
Enhancing Radiation Therapy Through Cherenkov Light-Activated Phototherapy.
Breast Neoplasms
Evaluating mitochondrial DNA in patients with breast cancer and benign breast disease.
Breast Neoplasms
Half-sandwich Ru(?6-p-cymene) complexes featuring pyrazole appended ligands: Synthesis, DNA binding and in vitro cytotoxicity.
Breast Neoplasms
Heterobimetallic Ru(ii)/Fe(ii) complexes as potent anticancer agents against breast cancer cells, inducing apoptosis through multiple targets.
Breast Neoplasms
Highly Promising Antitumor Agent of a Novel Platinum(II) Complex Bearing a Tetradentate Chelating Ligand.
Breast Neoplasms
Hyperactivation of mammalian target of rapamycin complex 1 (mTORC1) promotes breast cancer progression through enhancing glucose starvation-induced autophagy and Akt signaling.
Breast Neoplasms
Interplay between ShcA Signaling and PGC-1? Triggers Targetable Metabolic Vulnerabilities in Breast Cancer.
Breast Neoplasms
Longitudinal serum metabolomics evaluation of trastuzumab and everolimus combination as pre-operative treatment for HER-2 positive breast cancer patients.
Breast Neoplasms
Lymphangioleiomyomatosis Biomarkers Linked to Lung Metastatic Potential and Cell Stemness.
Breast Neoplasms
microRNA regulation of mammalian target of rapamycin expression and activity controls estrogen receptor function and RAD001 sensitivity.
Breast Neoplasms
Mitochondrial complex I activity and NAD+/NADH balance regulate breast cancer progression.
Breast Neoplasms
Mitochondrial complex I and V gene polymorphisms associated with breast cancer in mizo-mongloid population.
Breast Neoplasms
Mitochondrial dysfunction promotes breast cancer cell migration and invasion through HIF1? accumulation via increased production of reactive oxygen species.
Breast Neoplasms
Mitochondrial NADH dehydrogenase polymorphisms are associated with breast cancer in Poland.
Breast Neoplasms
mTORC1 is a target of nordihydroguaiaretic acid to prevent breast tumor growth in vitro and in vivo.
Breast Neoplasms
mTORC1/2 targeted by n-3 polyunsaturated fatty acids in the prevention of mammary tumorigenesis and tumor progression.
Breast Neoplasms
Multiomics integrative analysis reveals antagonistic roles of CBX2 and CBX7 in metabolic reprogramming of breast cancer.
Breast Neoplasms
NADH: ubiquinone oxidoreductase inhibitors block induction of ornithine decarboxylase activity in MCF-7 human breast cancer cells.
Breast Neoplasms
Nicotinamide phosphoribosyltransferase can affect metastatic activity and cell adhesive functions by regulating integrins in breast cancer.
Breast Neoplasms
Oxoplatin-B, a cisplatin-based platinum(IV) complex with photoactive BODIPY for mitochondria specific "chemo-PDT" activity.
Breast Neoplasms
Phospholipase D-mTOR requirement for the Warburg effect in human cancer cells.
Breast Neoplasms
Rapid Assessment of Mitochondrial Complex I Activity and Metabolic Phenotyping of Breast Cancer Cells by NAD(p)H Cytometry.
Breast Neoplasms
Rheb1-Independent Activation of mTORC1 in Mammary Tumors Occurs through Activating Mutations in mTOR.
Breast Neoplasms
Ruthenium(II) p-cymene complex bearing 2,2'-dipyridylamine targets caspase 3 deficient MCF-7 breast cancer cells without disruption of antitumor immune response.
Breast Neoplasms
Silver(I) complexes of 3-methoxy-4-hydroxybenzaldehyde thiosemicarbazones and triphenylphosphine: structural, cytotoxicity, and apoptotic studies.
Breast Neoplasms
Sirtuin 1-dependent resveratrol cytotoxicity and pro-differentiation activity on breast cancer cells.
Breast Neoplasms
STAT1 potentiates oxidative stress revealing a targetable vulnerability that increases phenformin efficacy in breast cancer.
Breast Neoplasms
Suppression of mitochondrial complex I influences cell metastatic properties.
Breast Neoplasms
Synthesis, crystal structure, and anti-breast cancer activity of a novel metal-porphyrinic complex [YK(TCPP)(OH)2·(solvents)x].
Breast Neoplasms
Synthesis, molecular structure, theoretical calculation, DNA/protein interaction and cytotoxic activity of manganese(III) complex with 8-hydroxyquinoline.
Breast Neoplasms
Targeting metabolic plasticity in breast cancer cells via mitochondrial complex I modulation.
Breast Neoplasms
The combination of rapamycin and resveratrol blocks autophagy and induces apoptosis in breast cancer cells.
Breast Neoplasms
The mammalian target of rapamycin complex 1 (mTORC1) in breast cancer: the impact of oestrogen receptor and HER2 pathways.
Breast Neoplasms
The mechanistic target for rapamycin pathway is related to the phosphorylation score for estrogen receptor-? in human breast tumors in vivo.
Breast Neoplasms
The variation of mitochondrial NADH dehydrogenase subunit 4 (mtND4) and molecular dynamics simulation of SNPs among Iranian women with breast cancer.
Breast Neoplasms
Upregulation of GRIM-19 inhibits the growth and invasion of human breast cancer cells.
Burkitt Lymphoma
Inhibition of monocarboxyate transporter 1 by AZD3965 as a novel therapeutic approach for diffuse large B-cell lymphoma and Burkitt lymphoma.
Bursitis
Mitochondrial complex I and III mutations and neutral-lipid storage in activated mononuclear macrophages and neutrophils: a case presenting with necrotizing myopathy, poikiloderma atrophicans vasculare, and xanthogranulomatous bursitis.
Carcinogenesis
A combined approach against tumorigenesis using glucose deprivation and mitochondrial complex 1 inhibition by rotenone.
Carcinogenesis
A heteroplasmic, not homoplasmic, mitochondrial DNA mutation promotes tumorigenesis via alteration in reactive oxygen species generation and apoptosis.
Carcinogenesis
Akt-dependent Skp2 mRNA translation is required for exiting contact inhibition, oncogenesis, and adipogenesis.
Carcinogenesis
AKT-mediated regulation of chromatin ubiquitylation and tumorigenesis through Mel18 phosphorylation.
Carcinogenesis
Antisense insulin-like growth factor I transferred into a rat hepatoma cell line inhibits tumorigenesis by modulating major histocompatibility complex I cell surface expression.
Carcinogenesis
Bile acid exposure up-regulates tuberous sclerosis complex 1/mammalian target of rapamycin pathway in Barrett's-associated esophageal adenocarcinoma.
Carcinogenesis
Conditional disruption of rictor demonstrates a direct requirement for mTORC2 in skin tumor development and continued growth of established tumors.
Carcinogenesis
Curcumin represses mTORC1 signaling in Caco-2 cells by a two-sided mechanism involving the loss of IRS-1 and activation of AMPK.
Carcinogenesis
Decreased expression of GRIM-19 by DNA hypermethylation promotes aerobic glycolysis and cell proliferation in head and neck squamous cell carcinoma.
Carcinogenesis
Down-regulation of GRIM-19 promotes the growth and migration of human glioma cells.
Carcinogenesis
Downregulation of microRNA-425-5p suppresses cervical cancer tumorigenesis by targeting AIFM1.
Carcinogenesis
Elevation of n-3/n-6 PUFAs ratio suppresses mTORC1 and prevents colorectal carcinogenesis associated with APC mutation.
Carcinogenesis
Gene associated with retinoid-interferon-induced mortality-19 suppresses growth of lung adenocarcinoma tumor in vitro and in vivo.
Carcinogenesis
GRIM-19 inhibition induced autophagy through activation of ERK and HIF-1? not STAT3 in Hela cells.
Carcinogenesis
GRIM-19 opposes reprogramming of glioblastoma cell metabolism via HIF1? destabilization.
Carcinogenesis
GRIM-19 Restores Cervical Cancer Cell Senescence by Repressing hTERT Transcription.
Carcinogenesis
Hepatitis B Virus X Protein Upregulates mTOR Signaling through IKK? to Increase Cell Proliferation and VEGF Production in Hepatocellular Carcinoma.
Carcinogenesis
JNK Signaling Promotes Intestinal Tumorigenesis Through Activation of mTOR Complex 1 in Apc?716 Mice.
Carcinogenesis
Knockdown BMI1 expression inhibits proliferation and invasion in human bladder cancer T24 cells.
Carcinogenesis
LPS promotes HBO1 stability via USP25 to modulate inflammatory gene transcription in THP-1 cells.
Carcinogenesis
Metformin inhibits mitochondrial complex I of cancer cells to reduce tumorigenesis.
Carcinogenesis
miR-125b-5p/STAT3 Pathway Regulated by mTORC1 Plays a Critical Role in Promoting Cell Proliferation and Tumor Growth.
Carcinogenesis
Mitochondrial DNA somatic mutations (point mutations and large deletions) and mitochondrial DNA variants in human thyroid pathology: a study with emphasis on Hürthle cell tumors.
Carcinogenesis
Mitochondrial GRIM-19 as a potential therapeutic target for STAT3-dependent carcinogenesis of gastric cancer.
Carcinogenesis
Mitochondrial GRIM-19 deficiency facilitates gastric cancer metastasis through oncogenic ROS-NRF2-HO-1 axis via a NRF2-HO-1 loop.
Carcinogenesis
Mitochondrial respiratory complex I dysfunction promotes tumorigenesis through ROS alteration and AKT activation.
Carcinogenesis
Monoallelic loss of tumor suppressor GRIM-19 promotes tumorigenesis in mice.
Carcinogenesis
Polycomb Repressor Complex 1 Member, BMI1 Contributes to Urothelial Tumorigenesis through p16-Independent Mechanisms.
Carcinogenesis
SIRT3 aggravates metformin-induced energy stress and apoptosis in ovarian cancer cells.
Carcinogenesis
Somatic complex I disruptive mitochondrial DNA mutations are modifiers of tumorigenesis that correlate with low genomic instability in pituitary adenomas.
Carcinogenesis
Suppression of mTOR via Akt dependent and independent mechanisms in selenium treated colon cancer cells: involvement of AMPK{alpha}1.
Carcinogenesis
[Correlations of GRIM-19 and its target gene product STAT3 to malignancy of human colorectal carcinoma]
Carcinoid Tumor
A phase 2 study of an oral mTORC1/mTORC2 kinase inhibitor (CC-223) for non-pancreatic neuroendocrine tumors with or without carcinoid symptoms.
Carcinoid Tumor
Non-toxic fragment of botulinum neurotoxin type A and monomethyl auristatin E conjugate for targeted therapy for neuroendocrine tumors.
Carcinoma
A bifunctional platinum(II) complex capable of intercalation and hydrogen-bonding interactions with DNA: binding studies and cytotoxicity.
Carcinoma
A dual-targeting, apoptosis-inducing organometallic half-sandwich iridium anticancer complex.
Carcinoma
A new Cu(II)-O-Carvacrotinate complex: Synthesis, characterization and biological activity.
Carcinoma
A Phase 1 Study of Everolimus + Weekly Cisplatin + Intensity Modulated Radiation Therapy in Head-and-Neck Cancer.
Carcinoma
A phase I study of temsirolimus and bryostatin-1 in patients with metastatic renal cell carcinoma and soft tissue sarcoma.
Carcinoma
A phase I study of temsirolimus plus carboplatin plus paclitaxel for patients with recurrent or metastatic (R/M) head and neck squamous cell cancer (HNSCC).
Carcinoma
A Phase Ib Study of BEZ235, a Dual Inhibitor of Phosphatidylinositol 3-Kinase (PI3K) and Mammalian Target of Rapamycin (mTOR), in Patients With Advanced Renal Cell Carcinoma.
Carcinoma
A proteomic analysis reveals the loss of expression of the cell death regulatory gene GRIM-19 in human renal cell carcinomas.
Carcinoma
A Rhodium(III)-Based Inhibitor of Lysine-Specific Histone Demethylase 1 as an Epigenetic Modulator in Prostate Cancer Cells.
Carcinoma
AIF suppresses chemical stress-induced apoptosis and maintains the transformed state of tumor cells.
Carcinoma
Arecoline downregulates levels of p21 and p27 through the reactive oxygen species/mTOR complex 1 pathway and may contribute to oral squamous cell carcinoma.
Carcinoma
BAY 87-2243, a novel inhibitor of hypoxia-induced gene activation, improves local tumor control after fractionated irradiation in a schedule-dependent manner in head and neck human xenografts.
Carcinoma
Biological evaluation of a cytotoxic 2-substituted benzimidazole copper(II) complex: DNA damage, antiproliferation and apoptotic induction activity in human cervical cancer cells.
Carcinoma
CBX7 and HMGA1b proteins act in opposite way on the regulation of the SPP1 gene expression.
Carcinoma
cis-[PtCl2(4,7-H-5-methyl-7-oxo[1,2,4]triazolo[1,5-a]pyrimidine)2]: a sterically restrictive new cisplatin analogue. Reaction kinetics with model nucleobases, DNA interaction studies, antitumor activity, and structure-activity relationships.
Carcinoma
Copper(ii) complexes of functionalized 2,2':6',2''-terpyridines and 2,6-di(thiazol-2-yl)pyridine: structure, spectroscopy, cytotoxicity and catalytic activity.
Carcinoma
Cytotoxic effect of thiacarbocyanine dyes on human colon carcinoma cells and inhibition of bovine heart mitochondrial NADH-ubiquinone reductase activity via a rotenone-type mechanism by two of the dyes.
Carcinoma
D-loop Mutations in Renal Cell Carcinoma Improve Predictive Accuracy for Cancer-Related Death by Integrating with Mutations in the NADH Dehydrogenase Subunit 1 Gene.
Carcinoma
Decreased expression of GRIM-19 and its association with high-risk HPV infection in cervical squamous intraepithelial neoplasias and cancer.
Carcinoma
Decreased expression of GRIM-19 by DNA hypermethylation promotes aerobic glycolysis and cell proliferation in head and neck squamous cell carcinoma.
Carcinoma
Detection of mitochondrial genome depletion by a novel cDNA in renal cell carcinoma.
Carcinoma
Development of a Long-Lived Luminescence Probe for Visualizing ?-Galactosidase in Ovarian Carcinoma Cells.
Carcinoma
Differential effects of all-trans retinoic acid on the growth of human keratinocytes and mouth carcinoma epidermoid cultures. Involvement of GRIM-19 and complex I of the respiratory chain.
Carcinoma
Differential expression of organic cation transporter OCT-3 in oral premalignant and malignant lesions: potential implications in the antineoplastic effects of metformin.
Carcinoma
Differential expression of the mitochondrial gene cytochrome oxidase II in benign and malignant breast tissue.
Carcinoma
Doubly end-on azido bridged mixed-valence cobalt trinuclear complex: Spectral study, VTM, inhibitory effect and antimycobacterial activity on human carcinoma and tuberculosis cells.
Carcinoma
Down-regulation of GRIM-19 is associated with STAT3 overexpression in breast carcinomas.
Carcinoma
Effective use of everolimus as salvage chemotherapy for ovarian clear cell carcinoma: a case report.
Carcinoma
Epidermal growth factor receptor activity upregulates lactate dehydrogenase A expression, lactate dehydrogenase activity, and lactate secretion in cultured IB3-1 cystic fibrosis lung epithelial cells.
Carcinoma
Fisetin inhibits cellular proliferation and induces mitochondria-dependent apoptosis in human gastric cancer cells.
Carcinoma
GRIM-19 deficiency promotes clear cell renal cell carcinoma progression and is associated with high TNM stage and Fuhrman grade.
Carcinoma
GRIM-19 represses the proliferation and invasion of cutaneous squamous cell carcinoma cells associated with downregulation of STAT3 signaling.
Carcinoma
Heightening Energetic Stress Selectively Targets LKB1-Deficient Non-Small Cell Lung Cancers.
Carcinoma
Identification of NADH dehydrogenase 1 alpha subcomplex 5 capable to transform murine fibroblasts and overexpressed in human cervical carcinoma cell lines.
Carcinoma
In vitro and in vivo anticancer activity of copper(I) complexes with homoscorpionate tridentate tris(pyrazolyl)borate and auxiliary monodentate phosphine ligands.
Carcinoma
Inhibition of electron flow through complex I of the mitochondrial respiratory chain of Ehrlich ascites carcinoma cells by methylglyoxal.
Carcinoma
Investigation of in vitro anticancer and DNA strap interactions in live cells using carboplatin type Cu(II) and Zn(II) metalloinsertors.
Carcinoma
Major histocompatibility complex I upregulation in clear cell renal cell carcinoma is associated with increased survival.
Carcinoma
Metformin Clinical Trial in HPV+ and HPV- Head and Neck Squamous Cell Carcinoma: Impact on Cancer Cell Apoptosis and Immune Infiltrate.
Carcinoma
Microsatellite instability in the mitochondrial DNA of colorectal carcinomas: evidence for mismatch repair systems in mitochondrial genome.
Carcinoma
Morphoproteomics and biomedical analytics confirm the mTORC2/Akt pathway as a resistance signature and activated ERK and STAT3 as concomitant prosurvival/antiapoptotic pathways in metastatic renal cell carcinoma (RCC) progressing on rapalogs: Pathogenesis.
Carcinoma
Norcantharidin counteracts acquired everolimus resistance in renal cell carcinoma by dual inhibition of mammalian target of rapamycin complex 1 and complex 2 pathways in Vitro.
Carcinoma
Novel Palladium(II) Complexes that Influence Prominin-1/CD133 Expression and Stem Cell Factor Release in Tumor Cells.
Carcinoma
Novel therapeutic roles of MC-4 in combination with everolimus against advanced renal cell carcinoma by dual targeting of Akt/pyruvate kinase muscle isozyme M2 and mechanistic target of rapamycin complex 1 pathways.
Carcinoma
OCIAD1 Controls Electron Transport Chain Complex I Activity to Regulate Energy Metabolism in Human Pluripotent Stem Cells.
Carcinoma
Oncogenic properties of a spermatogenic meiotic variant of fer kinase expressed in somatic cells.
Carcinoma
p53 Deletion or Hotspot Mutations Enhance mTORC1 Activity by Altering Lysosomal Dynamics of TSC2 and Rheb.
Carcinoma
Plasmid-based Stat3-specific siRNA and GRIM-19 inhibit the growth of thyroid cancer cells in vitro and in vivo.
Carcinoma
Possible Involvement of Persistent Activity of the Mammalian Target of Rapamycin Pathway in the Cisplatin Resistance of AFP-Producing Gastric Cancer Cells.
Carcinoma
Role of NADH Dehydrogenase (Ubiquinone) 1 Alpha Subcomplex 4-Like 2 in Clear Cell Renal Cell Carcinoma.
Carcinoma
Somatic and germline mutation in GRIM-19, a dual function gene involved in mitochondrial metabolism and cell death, is linked to mitochondrion-rich (Hurthle cell) tumours of the thyroid.
Carcinoma
Study of the biological effects and DNA damage exerted by a new dipalladium-Hmtpo complex on human cancer cells.
Carcinoma
Systematic Expression Analysis of Mitochondrial Complex I Identifies NDUFS1 as a Biomarker in Clear-Cell Renal-Cell Carcinoma.
Carcinoma
Tumor cell-derived secretory factor downregulates Semaphorin-3a in osteoblasts by activating mammalian target of rapamycin pathway.
Carcinoma
Tumor Microenvironment Modulates Immunological Outcomes of Myeloid Cells with mTORC1 Disruption.
Carcinoma
Upregulation of GRIM-19 suppresses the growth of oral squamous cell carcinoma in vitro and in vivo.
Carcinoma
Von Hippel-Lindau tumor suppressor pathways & corresponding therapeutics in kidney cancer.
Carcinoma, Ehrlich Tumor
Electron-transferring enzymes in the plasma membrane of the Ehrlich ascites tumor cell.
Carcinoma, Ehrlich Tumor
Oxidative stress-mediated cytotoxicity of Endosulfan is causally linked to the inhibition of NADH dehydrogenase and Na+, K+-ATPase in Ehrlich ascites tumor cells.
Carcinoma, Ehrlich Tumor
Purification and characterization of a plasma membrane ferricyanide-utilizing NADH dehydrogenase from Ehrlich tumour cells.
Carcinoma, Ehrlich Tumor
[Influence of local anesthetics and narcotics on the energy metabolism of Ehrlich ascites tumor cells.]
Carcinoma, Hepatocellular
A platinum(II) complex of liriodenine from traditional Chinese medicine (TCM): Cell cycle arrest, cell apoptosis induction and telomerase inhibition activity via G-quadruplex DNA stabilization.
Carcinoma, Hepatocellular
A two-dimensional electrophoresis preliminary approach to human hepatocarcinoma differentiation induced by PPAR-agonists.
Carcinoma, Hepatocellular
Antisense insulin-like growth factor I transferred into a rat hepatoma cell line inhibits tumorigenesis by modulating major histocompatibility complex I cell surface expression.
Carcinoma, Hepatocellular
AP1S3 is required for hepatitis C virus infection by stabilizing E2 protein.
Carcinoma, Hepatocellular
Chronic activation of mTOR complex 1 is sufficient to cause hepatocellular carcinoma in mice.
Carcinoma, Hepatocellular
Defects in mTORC1 Network and mTORC1-STAT3 Pathway Crosstalk Contributes to Non-inflammatory Hepatocellular Carcinoma.
Carcinoma, Hepatocellular
Depletion of GRIM-19 accelerates hepatocellular carcinoma invasion via inducing EMT and loss of contact inhibition.
Carcinoma, Hepatocellular
Dinuclear Pt(ii)-bisphosphonate complexes: a scaffold for multinuclear or different oxidation state platinum drugs.
Carcinoma, Hepatocellular
Down-regulation of a Mitochondrial Micropeptide MPM Promotes Hepatoma Metastasis by Enhancing Mitochondrial Complex I Activity.
Carcinoma, Hepatocellular
Downregulation of GRIM-19 is associated with hyperactivation of p-STAT3 in hepatocellular carcinoma.
Carcinoma, Hepatocellular
Effects of new tetrahydroquinoline-isoxazole hybrides on bioenergetics of hepatocarcinoma Hep-G2 cells and rat liver mitochondria.
Carcinoma, Hepatocellular
Enhanced mLST8 Expression Correlates with Tumor Progression in Hepatocellular Carcinoma.
Carcinoma, Hepatocellular
Fluorescence and HPLC Detection of Hydroxyl Radical by a Rhodamine-Nitroxide Probe and its Application in Cell Imaging.
Carcinoma, Hepatocellular
Hepatitis B Virus X Protein Upregulates mTOR Signaling through IKK? to Increase Cell Proliferation and VEGF Production in Hepatocellular Carcinoma.
Carcinoma, Hepatocellular
Impairment of the mitochondrial respiratory chain activity in diethylnitrosamine-induced rat hepatomas: possible involvement of oxygen free radicals.
Carcinoma, Hepatocellular
Increased steady-state levels of several mitochondrial and nuclear gene transcripts in rat hepatoma with a low content of mitochondria.
Carcinoma, Hepatocellular
Knockdown of THOC1 reduces the proliferation of hepatocellular carcinoma and increases the sensitivity to cisplatin.
Carcinoma, Hepatocellular
Liver damage, inflammation, and enhanced tumorigenesis after persistent mTORC1 inhibition.
Carcinoma, Hepatocellular
Macrophage Raptor Deficiency-Induced Lysosome Dysfunction Exacerbates Nonalcoholic Steatohepatitis.
Carcinoma, Hepatocellular
Mechanistic target of rapamycin complex 1 (mTORC1) is an essential mediator of metabolic and mitogenic effects of FGF19 in hepatoma cells.
Carcinoma, Hepatocellular
Metformin requires 4E-BPs to induce apoptosis and repress translation of Mcl-1 in hepatocellular carcinoma cells.
Carcinoma, Hepatocellular
Metformin suppresses glucose-6-phosphatase expression by a complex I inhibition and AMPK activation-independent mechanism.
Carcinoma, Hepatocellular
Mitochondrial transcription factor A and its downstream targets are up-regulated in a rat hepatoma.
Carcinoma, Hepatocellular
Novel candidate biomarkers of origin recognition complex 1, 5 and 6 for survival surveillance in patients with hepatocellular carcinoma.
Carcinoma, Hepatocellular
Overexpression of apoptosis-inducing factor mitochondrion-associated 1 (AIFM1) induces apoptosis by promoting the transcription of caspase3 and DRAM in hepatoma cells.
Carcinoma, Hepatocellular
Overexpression of GRIM-19, a mitochondrial respiratory chain complex I protein, suppresses hepatocellular carcinoma growth.
Carcinoma, Hepatocellular
Proliferating effect of orotic acid through mTORC1 activation mediated by negative regulation of AMPK in SK-Hep1 hepatocellular carcinoma cells.
Carcinoma, Hepatocellular
Proteomic analysis of laser capture microdissected focal lesions in a rat model of progenitor marker-positive hepatocellular carcinoma.
Carcinoma, Hepatocellular
Radiosensitization by inhibiting complex I activity in human hepatoma HepG2 cells to X-ray radiation.
Carcinoma, Hepatocellular
Respiratory chain-linked NADH dehydrogenase. Mechanisms of assembly.
Carcinoma, Hepatocellular
Silencing non-SMC chromosome-associated polypeptide G inhibits proliferation and induces apoptosis in hepatocellular carcinoma cells.
Carcinoma, Hepatocellular
SQSTM1/p62 activates NFE2L2/NRF2 via ULK1-mediated autophagic KEAP1 degradation and protects mouse liver from lipotoxicity.
Carcinoma, Hepatocellular
Synergistic effects of co-expression plasmid?based ADAM10-specific siRNA and GRIM-19 on hepatocellular carcinoma in vitro and in vivo.
Carcinoma, Hepatocellular
Synthesis and biological evaluation of novel platinum complexes of imidazolyl-containing bisphosphonates as potential anticancer agents.
Carcinoma, Hepatocellular
The double-edged sword of MTOR in autophagy deficiency induced-liver injury and tumorigenesis.
Carcinoma, Hepatocellular
The ménage à trois of autophagy, lipid droplets and liver disease.
Carcinoma, Hepatocellular
The rapid activation of protein synthesis by growth hormone requires signaling through mTOR.
Carcinoma, Hepatocellular
Yes-associated protein 1 and transcriptional coactivator with PDZ-binding motif activate the mammalian target of rapamycin complex 1 pathway by regulating amino acid transporters in hepatocellular carcinoma.
Carcinoma, Hepatocellular
Zinc (II) complex with a cationic Schiff base ligand: synthesis, characterization, and biological studies.
Carcinoma, Lewis Lung
In vitro and in vivo anticancer activity of copper(I) complexes with homoscorpionate tridentate tris(pyrazolyl)borate and auxiliary monodentate phosphine ligands.
Carcinoma, Lewis Lung
Tumor cell-derived secretory factor downregulates Semaphorin-3a in osteoblasts by activating mammalian target of rapamycin pathway.
Carcinoma, Lewis Lung
Tumor Microenvironment Modulates Immunological Outcomes of Myeloid Cells with mTORC1 Disruption.
Carcinoma, Non-Small-Cell Lung
Association of predicted pathogenic mutations in mitochondrial ND genes with distant metastasis in NSCLC and colon cancer.
Carcinoma, Non-Small-Cell Lung
Heightening Energetic Stress Selectively Targets LKB1-Deficient Non-Small Cell Lung Cancers.
Carcinoma, Non-Small-Cell Lung
HORMAD1 is a negative prognostic indicator in lung adenocarcinoma and specifies resistance to oxidative and genotoxic stress.
Carcinoma, Non-Small-Cell Lung
MCT4 is induced by metastasis-enhancing pathogenic mitochondrial NADH dehydrogenase gene mutations and can be a therapeutic target.
Carcinoma, Non-Small-Cell Lung
PAQR3 suppresses the growth of non-small cell lung cancer cells via modulation of EGFR-mediated autophagy.
Carcinoma, Non-Small-Cell Lung
Programmed death ligand 1-associated antitumor immunity in early-stage non-small cell lung cancer: Expression of major histocompatibility complex I as a prerequisite.
Carcinoma, Non-Small-Cell Lung
The anti-cancer drug ABTL0812 induces ER stress-mediated cytotoxic autophagy by increasing dihydroceramide levels in cancer cells.
Carcinoma, Non-Small-Cell Lung
[Expression and clinical significance of GRIM-19 in non-small cell lung cancer.]
Carcinoma, Papillary
Mitochondrial DNA somatic mutations (point mutations and large deletions) and mitochondrial DNA variants in human thyroid pathology: a study with emphasis on Hürthle cell tumors.
Carcinoma, Papillary
mTORC1-driven accumulation of the oncometabolite fumarate as a potential critical step in renal cancer progression.
Carcinoma, Papillary
Somatic and germline mutation in GRIM-19, a dual function gene involved in mitochondrial metabolism and cell death, is linked to mitochondrion-rich (Hurthle cell) tumours of the thyroid.
Carcinoma, Renal Cell
A phase I study of temsirolimus and bryostatin-1 in patients with metastatic renal cell carcinoma and soft tissue sarcoma.
Carcinoma, Renal Cell
A Phase Ib Study of BEZ235, a Dual Inhibitor of Phosphatidylinositol 3-Kinase (PI3K) and Mammalian Target of Rapamycin (mTOR), in Patients With Advanced Renal Cell Carcinoma.
Carcinoma, Renal Cell
A proteomic analysis reveals the loss of expression of the cell death regulatory gene GRIM-19 in human renal cell carcinomas.
Carcinoma, Renal Cell
D-loop Mutations in Renal Cell Carcinoma Improve Predictive Accuracy for Cancer-Related Death by Integrating with Mutations in the NADH Dehydrogenase Subunit 1 Gene.
Carcinoma, Renal Cell
Detection of mitochondrial genome depletion by a novel cDNA in renal cell carcinoma.
Carcinoma, Renal Cell
GRIM-19 deficiency promotes clear cell renal cell carcinoma progression and is associated with high TNM stage and Fuhrman grade.
Carcinoma, Renal Cell
Identification of alternatively spliced GRIM-19 mRNA in kidney cancer tissues.
Carcinoma, Renal Cell
Major histocompatibility complex I upregulation in clear cell renal cell carcinoma is associated with increased survival.
Carcinoma, Renal Cell
Morphoproteomics and biomedical analytics confirm the mTORC2/Akt pathway as a resistance signature and activated ERK and STAT3 as concomitant prosurvival/antiapoptotic pathways in metastatic renal cell carcinoma (RCC) progressing on rapalogs: Pathogenesis.
Carcinoma, Renal Cell
Norcantharidin counteracts acquired everolimus resistance in renal cell carcinoma by dual inhibition of mammalian target of rapamycin complex 1 and complex 2 pathways in Vitro.
Carcinoma, Renal Cell
Novel therapeutic roles of MC-4 in combination with everolimus against advanced renal cell carcinoma by dual targeting of Akt/pyruvate kinase muscle isozyme M2 and mechanistic target of rapamycin complex 1 pathways.
Carcinoma, Renal Cell
Reductive carboxylation supports growth in tumour cells with defective mitochondria.
Carcinoma, Renal Cell
Role of NADH Dehydrogenase (Ubiquinone) 1 Alpha Subcomplex 4-Like 2 in Clear Cell Renal Cell Carcinoma.
Carcinoma, Renal Cell
The effect of a gene associated with retinoid-interferon-induced mortality 19 (GRIM-19) on STAT3-induced gene expression in renal carcinoma.
Carcinoma, Renal Cell
Von Hippel-Lindau tumor suppressor pathways & corresponding therapeutics in kidney cancer.
Carcinoma, Squamous Cell
A Phase 1 Study of Everolimus + Weekly Cisplatin + Intensity Modulated Radiation Therapy in Head-and-Neck Cancer.
Carcinoma, Squamous Cell
A phase I study of temsirolimus plus carboplatin plus paclitaxel for patients with recurrent or metastatic (R/M) head and neck squamous cell cancer (HNSCC).
Carcinoma, Squamous Cell
BAY 87-2243, a novel inhibitor of hypoxia-induced gene activation, improves local tumor control after fractionated irradiation in a schedule-dependent manner in head and neck human xenografts.
Carcinoma, Squamous Cell
Decreased expression of GRIM-19 and its association with high-risk HPV infection in cervical squamous intraepithelial neoplasias and cancer.
Carcinoma, Squamous Cell
Decreased expression of GRIM-19 by DNA hypermethylation promotes aerobic glycolysis and cell proliferation in head and neck squamous cell carcinoma.
Carcinoma, Squamous Cell
Differential expression of organic cation transporter OCT-3 in oral premalignant and malignant lesions: potential implications in the antineoplastic effects of metformin.
Carcinoma, Squamous Cell
GRIM-19 represses the proliferation and invasion of cutaneous squamous cell carcinoma cells associated with downregulation of STAT3 signaling.
Carcinoma, Squamous Cell
Metformin Clinical Trial in HPV+ and HPV- Head and Neck Squamous Cell Carcinoma: Impact on Cancer Cell Apoptosis and Immune Infiltrate.
Carcinoma, Squamous Cell
Monoallelic loss of tumor suppressor GRIM-19 promotes tumorigenesis in mice.
Cardiomegaly
Blocking eukaryotic initiation factor 4F complex formation does not inhibit the mTORC1-dependent activation of protein synthesis in cardiomyocytes.
Cardiomegaly
Cardiolipin deficiency elevates susceptibility to a lipotoxic hypertrophic cardiomyopathy.
Cardiomegaly
Changes in cardiac substrate transporters and metabolic proteins mirror the metabolic shift in patients with aortic stenosis.
Cardiomegaly
Glutaredoxin-2 controls cardiac mitochondrial dynamics and energetics in mice, and protects against human cardiac pathologies.
Cardiomegaly
High incidence and variable clinical outcome of cardiac hypertrophy due to ACAD9 mutations in childhood.
Cardiomegaly
Impact of Intravenous Iron on Oxidative Stress and Mitochondrial Function in Experimental Chronic Kidney Disease.
Cardiomegaly
Maternal nutrient restriction alters gene expression in the ovine fetal heart.
Cardiomegaly
Mitochondrial complex I deficiency and cardiovascular diseases: current evidence and future directions.
Cardiomegaly
Prenatal programming: adverse cardiac programming by gestational testosterone excess.
Cardiomegaly
Rheb activates protein synthesis and growth in adult rat ventricular cardiomyocytes.
Cardiomyopathies
A high rate (20%-30%) of parental consanguinity in cytochrome-oxidase deficiency.
Cardiomyopathies
A mitochondrial encephalomyopathy with cardiomyopathy. A case revealing a defect of complex I in the respiratory chain.
Cardiomyopathies
Complex I deficiency due to selective loss of ndufs4 in the mouse heart results in severe hypertrophic cardiomyopathy.
Cardiomyopathies
Deficiency of subunits in heart mitochondrial NADH-ubiquinone oxidoreductase of a patient with mitochondrial encephalomyopathy and cardiomyopathy.
Cardiomyopathies
Depletion of adenine nucleotide translocator protein in heart mitochondria from doxorubicin-treated rats-Relevance for mitochondrial dysfunction.
Cardiomyopathies
Excessive formation of hydroxyl radicals and aldehydic lipid peroxidation products in cultured skin fibroblasts from patients with complex I deficiency.
Cardiomyopathies
Exome sequencing identifies mitochondrial alanyl-tRNA synthetase mutations in infantile mitochondrial cardiomyopathy.
Cardiomyopathies
Familial cardiomyopathy with cataracts and lactic acidosis: a defect in complex I (NADH-dehydrogenase) of the mitochondria respiratory chain.
Cardiomyopathies
Histiocytoid cardiomyopathy and microphthalmia with linear skin defects syndrome: phenotypes linked by truncating variants in NDUFB11.
Cardiomyopathies
Impairments in left ventricular mitochondrial bioenergetics precede overt cardiac dysfunction and remodelling in Duchenne muscular dystrophy.
Cardiomyopathies
Infantile familial cardiomyopathy due to mitochondrial complex I and IV associated deficiency.
Cardiomyopathies
Manganese superoxide dismutase protects mitochondrial complex I against adriamycin-induced cardiomyopathy in transgenic mice.
Cardiomyopathies
Mitigation of NADH: ubiquinone oxidoreductase deficiency by chronic Trolox treatment.
Cardiomyopathies
Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase.
Cardiomyopathies
Mitochondrial encoded NADH dehydrogenase 5 (MT-ND5) gene point mutation presents as late onset cardiomyopathy.
Cardiomyopathies
Mitochondrial myopathies with necrotizing encephalopathy of the Leigh type.
Cardiomyopathies
Mitochondrial respiratory chain complex I deficiency with clinical and biochemical features of long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.
Cardiomyopathies
Mitochondrials complex I activity is reduced in latent adriamycin-induced cardiomyopathy of rat.
Cardiomyopathies
MTO1-Deficient Mouse Model Mirrors the Human Phenotype Showing Complex I Defect and Cardiomyopathy.
Cardiomyopathies
Mutant NDUFV2 subunit of mitochondrial complex I causes early onset hypertrophic cardiomyopathy and encephalopathy.
Cardiomyopathies
Mutations in the accessory subunit NDUFB10 result in isolated complex I deficiency and illustrate the critical role of intermembrane space import for complex I holoenzyme assembly.
Cardiomyopathies
Mutations in the complex I NDUFS2 gene of patients with cardiomyopathy and encephalomyopathy.
Cardiomyopathies
Neonatal multiorgan failure due to ACAD9 mutation and complex I deficiency with mitochondrial hyperplasia in liver, cardiac myocytes, skeletal muscle, and renal tubules.
Cardiomyopathies
New familial mitochondrial encephalopathy with macrocephaly, cardiomyopathy, and complex I deficiency.
Cardiomyopathies
Novel mutations in KARS cause hypertrophic cardiomyopathy and combined mitochondrial respiratory chain defect.
Cardiomyopathies
Successful pregnancy in a patient with mitochondrial cardiomyopathy due to ACAD9 deficiency.
Cardiomyopathies
Successful treatment of infantile-onset ACAD9-related cardiomyopathy with a combination of sodium pyruvate, beta-blocker, and coenzyme Q10.
Cardiomyopathies
Tissue-specific splicing of an Ndufs6 gene-trap insertion generates a mitochondrial complex I deficiency-specific cardiomyopathy.
Cardiomyopathies
[A case of mitochondrial encephalomyopathy with cardiomyopathy due to decreased complex I and IV activities]
Cardiomyopathies
[Mitochondrial function and mitochondrial DNA in a series of 64 patients suspected of having mitochondrial myopathy]
Cardiomyopathy, Dilated
A NADH dehydrogenase ubiquinone flavoprotein is decreased in patients with dilated cardiomyopathy.
Cardiomyopathy, Dilated
Cardiomyopathy associated with neurologic disorders and mitochondrial phenotype.
Cardiomyopathy, Dilated
Metformin Reverses the Enhanced Myocardial SR/ER-Mitochondria Interaction and Impaired Complex I-Driven Respiration in Dystrophin-Deficient Mice.
Cardiomyopathy, Dilated
Mitochondrial dysfunction in myocardium obtained from clinically normal dogs, clinically normal anesthetized dogs, and dogs with dilated cardiomyopathy.
Cardiomyopathy, Hypertrophic
A homozygous splicing mutation in ELAC2 suggests phenotypic variability including intellectual disability with minimal cardiac involvement.
Cardiomyopathy, Hypertrophic
Assembly defects of multiple respiratory chain complexes in a child with cardiac hypertrophy associated with a novel ACAD9 mutation.
Cardiomyopathy, Hypertrophic
Cardiac mitochondrial dysfunction and DNA depletion in children with hypertrophic cardiomyopathy.
Cardiomyopathy, Hypertrophic
Cardiomyopathy associated with neurologic disorders and mitochondrial phenotype.
Cardiomyopathy, Hypertrophic
Complex I deficiency due to selective loss of ndufs4 in the mouse heart results in severe hypertrophic cardiomyopathy.
Cardiomyopathy, Hypertrophic
Diverse phenotype in patients with complex I deficiency due to mutations in NDUFB11.
Cardiomyopathy, Hypertrophic
ELAC2 Mutations Cause a Mitochondrial RNA Processing Defect Associated with Hypertrophic Cardiomyopathy.
Cardiomyopathy, Hypertrophic
Exome sequencing identifies complex I NDUFV2 mutations as a novel cause of Leigh syndrome.
Cardiomyopathy, Hypertrophic
Heteroplasmic mitochondrial DNA 3310 mutation in NADH dehydrogenase subunit 1 associated with type 2 diabetes, hypertrophic cardiomyopathy, and mental retardation in a single patient.
Cardiomyopathy, Hypertrophic
Hypertrophic cardiomyopathy with mitochondrial DNA depletion and respiratory enzyme defects.
Cardiomyopathy, Hypertrophic
Mitochondrial complex I activity is significantly decreased in a patient with maternally inherited type 2 diabetes mellitus and hypertrophic cardiomyopathy associated with mitochondrial DNA C3310T mutation: a cybrid study.
Cardiomyopathy, Hypertrophic
Mitochondrial targeting of human NADH dehydrogenase (ubiquinone) flavoprotein 2 (NDUFV2) and its association with early-onset hypertrophic cardiomyopathy and encephalopathy.
Cardiomyopathy, Hypertrophic
Mutant NDUFV2 subunit of mitochondrial complex I causes early onset hypertrophic cardiomyopathy and encephalopathy.
Cardiomyopathy, Hypertrophic
Mutation in mitochondrial ribosomal protein MRPS22 leads to Cornelia de Lange-like phenotype, brain abnormalities and hypertrophic cardiomyopathy.
Cardiomyopathy, Hypertrophic
Mutations in the mitochondrial complex I assembly factor NDUFAF1 cause fatal infantile hypertrophic cardiomyopathy.
Cardiomyopathy, Hypertrophic
OXPHOS defects and mitochondrial DNA mutations in cardiomyopathy.
Cardiomyopathy, Hypertrophic
Severe congenital lactic acidosis and hypertrophic cardiomyopathy caused by an intronic variant in NDUFB7.
Cardiomyopathy, Hypertrophic
[Complex I (NADH coenzyme-Q-reductase) deficiency, MELAS syndrome and hypertrophic cardiomyopathy]
Cardiotoxicity
A critical risk-benefit assessment argues against the use of anthracyclines in induction regimens for newly diagnosed childhood acute lymphoblastic leukemia.
Cardiotoxicity
BCNU-Induced gR2 DEFECT mediates S-glutathionylation OF Complex I and RESPIRATORY uncoupling in myocardium.
Cardiotoxicity
Cardioprotective roles of sestrin 1 and sestrin 2 against doxorubicin cardiotoxicity.
Cardiotoxicity
Reduction of oxygen by NADH/NADH dehydrogenase in the presence of adriamycin.
Cardiotoxicity
The exogenous NADH dehydrogenase of heart mitochondria is the key enzyme responsible for selective cardiotoxicity of anthracyclines.
Cardiovascular Diseases
Mitochondrial complex I deficiency and cardiovascular diseases: current evidence and future directions.
Cardiovascular Diseases
The reduction of NDUFC2 expression is associated with mitochondrial impairment in circulating mononuclear cells of patients with acute coronary syndrome.
Cardiovascular Diseases
Vascular effects of disrupting endothelial mTORC1 signaling in obesity.
Carney Complex
Carney complex 1 with PRKAR1A mutations manifesting as multiple repeated skin myxomas: A case report.
Carney Complex
Familial non-medullary thyroid carcinoma: clinico-pathological features, current knowledge and novelty regarding genetic risk factors.
Catalepsy
Linagliptin potentiates the effect of l-dopa on the behavioural, biochemical and immunohistochemical changes in experimentally-induced Parkinsonism: Role of toll-like receptor 4, TGF-?1, NF-?B and glucagon-like peptide 1.
Catalepsy
Mechanism of the neuroprotective role of coenzyme Q10 with or without L-dopa in rotenone-induced parkinsonism.
Catalepsy
Neuroprotective effect of EGb761(R) and low-dose whole-body ?-irradiationin a rat model of Parkinson's disease.
Catalepsy
Role of metabotropic glutamate receptors in the mechanisms of experimental parkinsonism development.
Cataract
A frameshift mitochondrial complex I gene mutation in a patient with dystonia and cataracts: is the mutation pathogenic?
Cataract
Bilateral infantile cataractogenesis in a patient with deficiency of complex I, a mitochondrial electron transport chain enzyme.
Cataract
Early-onset cataracts, spastic paraparesis, and ataxia caused by a novel mitochondrial tRNAGlu (MT-TE) gene mutation causing severe complex I deficiency: a clinical, molecular, and neuropathologic study.
Cataract
Excessive formation of hydroxyl radicals and aldehydic lipid peroxidation products in cultured skin fibroblasts from patients with complex I deficiency.
Cataract
Familial cardiomyopathy with cataracts and lactic acidosis: a defect in complex I (NADH-dehydrogenase) of the mitochondria respiratory chain.
Cataract
Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase.
Cataract
Mutations of RagA GTPase in mTORC1 Pathway Are Associated with Autosomal Dominant Cataracts.
Cell Transformation, Neoplastic
Sustained Early Disruption of Mitochondrial Function Contributes to Arsenic-Induced Prostate Tumorigenesis.
Central Nervous System Diseases
Major Histocompatibility Complex I Expression by Motor Neurons and Its Implication in Amyotrophic Lateral Sclerosis.
Central Nervous System Diseases
Neuronal MHC-I expression and its implications in synaptic function, axonal regeneration and Parkinson's and other brain diseases.
Cerebellar Ataxia
Cerebellar ataxia, neuropathy, hearing loss, and intellectual disability due to AIFM1 mutation.
Cerebellar Ataxia
Mutations in AIFM1 cause an X-linked childhood cerebellar ataxia partially responsive to riboflavin.
Cerebellar Ataxia
Sporadic heteroplasmic single 5.5 kb mitochondrial DNA deletion associated with cerebellar ataxia, hypogonadotropic hypogonadism, choroidal dystrophy, and mitochondrial respiratory chain complex I deficiency.
Cerebral Hemorrhage
SQR mediates therapeutic effects of H2S by targeting mitochondrial electron transport to induce mitochondrial uncoupling.
Cerebrovascular Disorders
Interaction between longevity-associated mitochondrial DNA 5178 C/A polymorphism and cigarette smoking on hematological parameters in Japanese men.
Cerebrovascular Disorders
The novel targets of DL-3-n-butylphthalide predicted by similarity ensemble approach in combination with molecular docking study.
Cestode Infections
Contribution of NADH dehydrogenase subunit I and cytochrome C oxidase subunit I sequences toward identifying a case of human coenuriasis in France.
Chagas Disease
Differential transcription profiles in Trypanosoma cruzi associated with clinical forms of Chagas disease: Maxicircle NADH dehydrogenase subunit 7 gene truncation in asymptomatic patient isolates.
Chagas Disease
Variation in Mitochondrial NADH Dehydrogenase Subunit 5 and NADH Dehydrogenase Subunit 4 Genes in the Chagas Disease Vector Triatoma infestans (Hemiptera: Reduviidae).
Charcot-Marie-Tooth Disease
A novel AIFM1 mutation expands the phenotype to an infantile motor neuron disease.
Charcot-Marie-Tooth Disease
A novel AIFM1 mutation in a Chinese family with X-linked Charcot-Marie-Tooth disease type 4.
Charcot-Marie-Tooth Disease
Mitochondrial complex I deficiency in GDAP1-related autosomal dominant Charcot-Marie-Tooth disease (CMT2K).
Charcot-Marie-Tooth Disease
Ultrastructural examination of skin biopsies may assist in diagnosing mitochondrial cytopathy when muscle biopsies yield negative results.
Chemical and Drug Induced Liver Injury
Superoxide anion production by the mitochondrial respiratory chain of hepatocytes of rats with experimental toxic hepatitis.
Chickenpox
Varicella Zoster Virus Downregulates Programmed Death Ligand 1 and Major Histocompatibility Complex 1 in Human Brain Vascular Adventitial Fibroblasts, Perineurial Cells and Lung Fibroblasts.
Cholera
cAMP-dependent phosphorylation of the nuclear encoded 18-kDa (IP) subunit of respiratory complex I and activation of the complex in serum-starved mouse fibroblast cultures.
Cholera
Inhibition of the sodium-translocating NADH-ubiquinone oxidoreductase [Na+-NQR] decreases cholera toxin production in Vibrio cholerae O1 at the late exponential growth phase.
Cholestasis
[Intrahepatic cholestasis due to mitochondrial respiratory chain complex I deficiency in a Chinese boy].
Cholestasis
[Morpho-functional changes in the liver and the possibility of their correction in the offspring of rats with cholestasis].
Cholestasis, Intrahepatic
[Intrahepatic cholestasis due to mitochondrial respiratory chain complex I deficiency in a Chinese boy].
Chordoma
Aberrant hyperactivation of akt and Mammalian target of rapamycin complex 1 signaling in sporadic chordomas.
Choriocarcinoma
Reactive oxygen species from mitochondria impacts trophoblast fusion and the production of endocrine hormones by syncytiotrophoblasts.
Choriocarcinoma
Regulation of Human Trophoblast GLUT3 Glucose Transporter by Mammalian Target of Rapamycin Signaling.
Colitis
Arctigenin functions as a selective agonist of estrogen receptor ? to restrict mTORC1 activation and consequent Th17 differentiation.
Colitis, Ulcerative
The mitochondrial negative regulator MCJ modulates the interplay between microbiota and the host during ulcerative colitis.
Colonic Neoplasms
Activation of AMP-activated protein kinase (AMPK) mediates plumbagin-induced apoptosis and growth inhibition in cultured human colon cancer cells.
Colonic Neoplasms
AIF suppresses chemical stress-induced apoptosis and maintains the transformed state of tumor cells.
Colonic Neoplasms
Association of predicted pathogenic mutations in mitochondrial ND genes with distant metastasis in NSCLC and colon cancer.
Colonic Neoplasms
Autophagy inhibition sensitizes WYE-354-induced anti-colon cancer activity in vitro and in vivo.
Colonic Neoplasms
Colorectal cancer cells refractory to anti-VEGF treatment are vulnerable to glycolytic blockade due to persistent impairment of mitochondria.
Colonic Neoplasms
Conjugation of a gold(iii) complex with vitamin B1 and chlorambucil derivatives: anticancer evaluation and mechanistic insights.
Colonic Neoplasms
Epibrassinolide activates AKT to trigger autophagy with polyamine metabolism in SW480 and DLD-1 colon cancer cell lines.
Colonic Neoplasms
Evaluation of catacholase mimicking activity and apoptosis in human colorectal carcinoma cell line by activating mitochondrial pathway of copper(II) complex coupled with 2-(quinolin-8-yloxy)(methyl)benzonitrile and 8-hydroxyquinoline.
Colonic Neoplasms
Growth-factor dependent expression of the translationally controlled tumour protein TCTP is regulated through the PI3-K/Akt/mTORC1 signalling pathway.
Colonic Neoplasms
Identification of an annonaceous acetogenin mimetic, AA005, as an AMPK activator and autophagy inducer in colon cancer cells.
Colonic Neoplasms
NADH dehydrogenase complex I is overexpressed in incipient metastatic murine colon cancer cells.
Colonic Neoplasms
Tumor suppressive role of sestrin2 during colitis and colon carcinogenesis.
Colonic Neoplasms
[Correlations of GRIM-19 and its target gene product STAT3 to malignancy of human colorectal carcinoma]
Colorectal Neoplasms
A heteroplasmic, not homoplasmic, mitochondrial DNA mutation promotes tumorigenesis via alteration in reactive oxygen species generation and apoptosis.
Colorectal Neoplasms
A new Cu(II)-O-Carvacrotinate complex: Synthesis, characterization and biological activity.
Colorectal Neoplasms
Are Wnt/?-Catenin and PI3K/AKT/mTORC1 Distinct Pathways in Colorectal Cancer?
Colorectal Neoplasms
Combination of mTOR and EGFR Kinase Inhibitors Blocks mTORC1 and mTORC2 Kinase Activity and Suppresses the Progression of Colorectal Carcinoma.
Colorectal Neoplasms
Differential regulation of mitochondrial complex I and oxidative stress based on metastatic potential of colorectal cancer cells.
Colorectal Neoplasms
Expression profiling of SCN8A and NDUFC2 genes in colorectal carcinoma.
Colorectal Neoplasms
GRIM-19 expression is a potent prognostic marker in colorectal cancer.
Colorectal Neoplasms
GRIM-19 inhibits proliferation and induces apoptosis in a p53-dependent manner in colorectal cancer cells through the SIRT7/PCAF/MDM2 axis.
Colorectal Neoplasms
GRIM-19 repressed hypoxia-induced invasion and EMT of colorectal cancer by repressing autophagy through inactivation of STAT3/HIF-1? signaling axis.
Colorectal Neoplasms
Highly Promising Antitumor Agent of a Novel Platinum(II) Complex Bearing a Tetradentate Chelating Ligand.
Colorectal Neoplasms
LYRM2 directly regulates complex I activity to support tumor growth in colorectal cancer by oxidative phosphorylation.
Colorectal Neoplasms
MicroRNA-451 regulates AMPK/mTORC1 signaling and fascin1 expression in HT-29 colorectal cancer cells.
Colorectal Neoplasms
Mutations in mitochondrial NADH dehydrogenase subunit 1 (mtND1) gene in colorectal carcinoma.
Colorectal Neoplasms
Piperine potentiates curcumin-mediated repression of mTORC1 signaling in human intestinal epithelial cells: implications for the inhibition of protein synthesis and TNF? signaling.
Colorectal Neoplasms
RAPTOR promotes colorectal cancer proliferation by inducing mTORC1 and upregulating ribosome assembly factor URB1.
Colorectal Neoplasms
[Correlations of GRIM-19 and its target gene product STAT3 to malignancy of human colorectal carcinoma]
Confusion
Fluxes of nicotinamide adenine dinucleotides through mitochondrial membranes in human cultured cells.
Confusion
Nicotinamide adenine dinucleotides permeate through mitochondrial membranes in human Epstein-Barr virus-transformed lymphocytes.
Coronary Disease
[The Effect of CYP4 F2 Polymorphism on Initial Warfarin Dose in Patients with Heart Valve Replacement].
Coronary Occlusion
Mitochondrial m-calpain opens the mitochondrial permeability transition pore in ischemia-reperfusion.
Cough
Indigenous knowledge of pastoralists on respiratory diseases of camels in northern Kenya.
COVID-19
Human gene polymorphisms and their possible impact on the clinical outcome of SARS-CoV-2 infection.
COVID-19
Lung transcriptome of a COVID-19 patient and systems biology predictions suggest impaired surfactant production which may be druggable by surfactant therapy.
Crohn Disease
Mitochondrial dysfunction in the gastrointestinal mucosa of children with autism: A blinded case-control study.
Cushing Syndrome
The relationship of mitochondrial dysfunction and the development of insulin resistance in Cushing's syndrome.
Cystadenoma
mTORC1-driven accumulation of the oncometabolite fumarate as a potential critical step in renal cancer progression.
Cystic Fibrosis
Disruption of interleukin-1? autocrine signaling rescues complex I activity and improves ROS levels in immortalized epithelial cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function.
Cystic Fibrosis
Mitochondrial NADH dehydrogenase in cystic fibrosis: enzyme kinetics in cultured fibroblasts.
Cystic Fibrosis
The Km of NADH dehydrogenase is decreased in mitochondria of cystic fibrosis cells.
Cystic Fibrosis
The mitochondrial Complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function.
Cysts
A first insight into the genotypes of Echinococcus granulosus from humans in Mongolia.
Cysts
Congenital lactic acidosis, cerebral cysts and pulmonary hypertension in an infant with FOXRED1 related complex 1 deficiency.
Cysts
Congenital lactic acidosis, cerebral cysts and pulmonary hypertension in an infant with FOXRED1 related complex I deficiency.
Cysts
Dispersal and molecular characterisation of the Echinococcus granulosus (Batsch, 1786) complex isolated from various intermediate hosts in the Calabria region, southern Italy.
Cysts
First molecular characterization of Echinococcus granulosus (sensu stricto) genotype 1 among cattle in Sudan.
Cysts
Genotyping of Echinococcus granulosus Isolates from Human in Khorasan Province, North-Eastern Iran.
Cysts
Molecular characterization of Echinococcus granulosus in south-eastern Romania: evidence of G1-G3 and G6-G10 complexes in humans.
Cysts
mTORC1-driven accumulation of the oncometabolite fumarate as a potential critical step in renal cancer progression.
Cysts
Multilocus sequence analysis of Echinococcus granulosus strains isolated from humans and animals in Iran.
Cysts
Myelopathy in Two Brothers with Respiratory Chain Disorder-Severe Complex 1 Deficiency with Atlantoaxial Dislocation and Long Spinal Arachnoid Cyst: A New Unreported Association.
Cysts
Polycomb group genes Psc and Su(z)2 maintain somatic stem cell identity and activity in Drosophila.
Cysts
Prevalence and Genotyping of Echinococcus Species from Livestock in Kajiado County, Kenya.
Cysts
Rapamycin and dexamethasone during pregnancy prevent tuberous sclerosis complex-associated cystic kidney disease.
Cysts
Targeting AMP-activated protein kinase (AMPK) for treatment of autosomal dominant polycystic kidney disease.
Cysts
The use of targeted genomic capture and massively parallel sequencing in diagnosis of Chinese Leukoencephalopathies.
Cytochrome-c Oxidase Deficiency
Analysis of the mitochondrial complex I-v enzyme activities of peripheral leukocytes in oxidative phosphorylation disorders.
Cytochrome-c Oxidase Deficiency
COA6 Facilitates Cytochrome c Oxidase Biogenesis as Thiol-reductase for Copper Metallochaperones in Mitochondria.
Cytochrome-c Oxidase Deficiency
Complex I function is defective in complex IV-deficient Caenorhabditis elegans.
Cytochrome-c Oxidase Deficiency
Deficiency of respiratory chain complex I is a common cause of Leigh disease.
Cytochrome-c Oxidase Deficiency
Identification of a new mtDNA mutation (14724G>A) associated with mitochondrial leukoencephalopathy.
Cytochrome-c Oxidase Deficiency
Increased risk for cardiorespiratory failure associated with the A3302G mutation in the mitochondrial DNA encoded tRNALeu(UUR) gene.
Cytochrome-c Oxidase Deficiency
Leigh syndrome with cytochrome-c oxidase deficiency and a single T insertion nt 5537 in the mitochondrial tRNATrp gene.
Cytochrome-c Oxidase Deficiency
Mitochondrial complex I mutations in Caenorhabditis elegans produce cytochrome c oxidase deficiency, oxidative stress and vitamin-responsive lactic acidosis.
Cytochrome-c Oxidase Deficiency
Mitochondrial DNA variants in inclusion body myositis characterized by deep sequencing.
Cytochrome-c Oxidase Deficiency
Mitochondrial myopathy and respiratory failure associated with a new mutation in the mitochondrial transfer ribonucleic acid glutamic acid gene.
Cytochrome-c Oxidase Deficiency
Mitochondrial Respiration Is Decreased in Rat Kidney Following Fetal Exposure to a MaternalLow-ProteinDiet.
Cytochrome-c Oxidase Deficiency
Noninvasive diagnostics of mitochondrial disorders in isolated lymphocytes with high resolution respirometry.
Cytochrome-c Oxidase Deficiency
Nonviability of cells with oxidative defects in galactose medium: a screening test for affected patient fibroblasts.
Cytochrome-c Oxidase Deficiency
Pathogenic SLIRP variants as a novel cause of autosomal recessive mitochondrial encephalomyopathy with complex I and IV deficiency.
Cytochrome-c Oxidase Deficiency
Pathological mechanisms underlying single large-scale mitochondrial DNA deletions.
Cytochrome-c Oxidase Deficiency
Predominant cerebellar volume loss as a neuroradiologic feature of pediatric respiratory chain defects.
Cytochrome-c Oxidase Deficiency
Prerequisites and strategies for prenatal diagnosis of respiratory chain deficiency in chorionic villi.
Cytochrome-c Oxidase Deficiency
Respiratory chain deficiency in a female with Aicardi-Goutières syndrome.
Cytochrome-c Oxidase Deficiency
[Clinical and investigative approaches in mitochondrial diseases. A review of 15 cases]
Cytochrome-c Oxidase Deficiency
[Mitochondrial function and mitochondrial DNA in a series of 64 patients suspected of having mitochondrial myopathy]
Cytomegalovirus Infections
Complex I binding by a virally encoded RNA regulates mitochondria-induced cell death.
Deafness
A novel AIFM1 mutation expands the phenotype to an infantile motor neuron disease.
Deafness
A novel AIFM1 mutation in a Chinese family with X-linked Charcot-Marie-Tooth disease type 4.
Deafness
A novel mutation in the mitochondrial tRNA(Pro) gene associated with late-onset ataxia, retinitis pigmentosa, deafness, leukoencephalopathy and complex I deficiency.
Deafness
Biochemical and genetic analysis of 3-methylglutaconic aciduria type IV: a diagnostic strategy.
Deafness
Novel Mutation in AIFM1 Gene Associated with X-Linked Deafness in a Moroccan Family.
Deafness
Severe multisystem pathology, metabolic acidosis, mitochondrial dysfunction, and early death associated with an X-linked AIFM1 variant.
Deafness
Spondyloepimetaphyseal dysplasia with neurodegeneration associated with AIFM1 mutation - a novel phenotype of the mitochondrial disease.
Deficiency Diseases
A novel NDUFV1 gene mutation in complex I deficiency in consanguineous siblings with brainstem lesions and Leigh syndrome.
Dehydration
Dehydration reaction of hydroxyl substituted alkenes and alkynes on the Ru(2)S(2) complex.
Dehydration
Enzyme histochemical demonstration of NADH dehydrogenase on resin-embedded tissue.
Dehydration
In the mitochondrial CMSII mutant of Nicotiana sylvestris photosynthetic activity remains higher than in the WT under persisting mild water stress
Dehydration
In the mitochondrial CMSII mutant of Nicotiana sylvestris photosynthetic activity remains higher than in the WT under persisting mild water stress.
Dehydration
Spin transitions in Fe(II) metallogrids modulated by substituents, counteranions, and solvents.
Delirium
5-HT(1A) receptor antagonist improves behavior performance of delirium rats through inhibiting PI3K/Akt/mTOR activation-induced NLRP3 activity.
Dementia
Complex I reductions in the nucleus basalis of Meynert in Lewy body dementia: the role of Lewy bodies.
Dementia
Dementia in Parkinson's disease is associated with enhanced mitochondrial complex I deficiency.
Dementia
Neurological complications of HIV-1-seropositive internal medicine inpatients in Kinshasa, Zaire.
Demyelinating Diseases
Inhibition of mitochondrial function induces an integrated stress response in oligodendroglia.
Dengue
HIV-1 Vpr protein impairs lysosome clearance causing SNCA/alpha-synuclein accumulation in neurons.
Dent Disease
Novel Fanconi renotubular syndromes provide insights in proximal tubule pathophysiology.
Dermatitis, Phototoxic
A photoactivatable Ru (II) complex bearing 2,9-diphenyl-1,10-phenanthroline: A potent chemotherapeutic drug inducing apoptosis in triple negative human breast adenocarcinoma cells.
Dermatitis, Phototoxic
Mitochondria and plasma membrane as targets of UVA-induced toxicity of neuroleptic drugs fluphenazine, perphenazine and thioridazine.
Diabetes Complications
Mitochondrial complex I deficiency and cardiovascular diseases: current evidence and future directions.
Diabetes Mellitus
Activation of mechanistic target of rapamycin complex 1: the common link between rheumatoid arthritis and diabetes mellitus.
Diabetes Mellitus
Essential role of glucokinase in the protection of pancreatic ? cells to the glucose energetic status.
Diabetes Mellitus
Mitochondrial complex I activity is significantly decreased in a patient with maternally inherited type 2 diabetes mellitus and hypertrophic cardiomyopathy associated with mitochondrial DNA C3310T mutation: a cybrid study.
Diabetes Mellitus
Mitochondrial complex I and V gene polymorphisms in type II diabetes mellitus among high risk Mizo-Mongoloid population, Northeast India.
Diabetes Mellitus
The GID ubiquitin ligase complex is a regulator of AMPK activity and organismal lifespan.
Diabetes Mellitus
Whole-blood transcriptome profiling reveals signatures of metformin and its therapeutic response.
Diabetes Mellitus
[Association of nt3434A→G Mutation in Mitochondrial DNA ND1 Gene with Diabetes Mellitus].
Diabetes Mellitus
[The Effect of CYP4 F2 Polymorphism on Initial Warfarin Dose in Patients with Heart Valve Replacement].
Diabetes Mellitus, Type 1
Inhibition of mitochondrial complex I may account for IDDM induced by intoxication with the rodenticide Vacor.
Diabetes Mellitus, Type 1
Insulin upregulates GRIM-19 and protects cardiac mitochondrial morphology in type 1 diabetic rats partly through PI3K/AKT signaling pathway.
Diabetes Mellitus, Type 1
mt-Nd2a modifies resistance against autoimmune Type 1 diabetes in NOD mice at the level of the pancreatic beta cell.
Diabetes Mellitus, Type 1
mt-Nd2a Suppresses Reactive Oxygen Species Production by Mitochondrial Complexes I and III.
Diabetes Mellitus, Type 1
Normal to enhanced intrinsic mitochondrial respiration in skeletal muscle of middle- to older-aged women and men with uncomplicated type 1 diabetes.
Diabetes Mellitus, Type 1
Temporal evolution of cardiac mitochondrial dysfunction in a type 1 diabetes model. Mitochondrial complex I impairment, and H2O2 and NO productions as early subcellular events.
Diabetes Mellitus, Type 1
The prevalence of mitochondrial gene mutations in childhood diabetes in Japan.
Diabetes Mellitus, Type 2
A Low-Protein Diet for Diabetic Kidney Disease: Its Effect and Molecular Mechanism, an Approach from Animal Studies.
Diabetes Mellitus, Type 2
Cross-talks via mTORC2 can explain enhanced activation in response to insulin in diabetic patients.
Diabetes Mellitus, Type 2
Differential expression of organic cation transporter OCT-3 in oral premalignant and malignant lesions: potential implications in the antineoplastic effects of metformin.
Diabetes Mellitus, Type 2
Drp1-mediated mitochondrial abnormalities link to synaptic injury in diabetes model.
Diabetes Mellitus, Type 2
Enhanced liver but not muscle OXPHOS in diabetes and reduced glucose output by complex I inhibition.
Diabetes Mellitus, Type 2
Essential role of glucokinase in the protection of pancreatic ? cells to the glucose energetic status.
Diabetes Mellitus, Type 2
Global metabolite profiling of mice with high-fat diet-induced obesity chronically treated with AMPK activators R118 or metformin reveals tissue-selective alterations in metabolic pathways.
Diabetes Mellitus, Type 2
Heteroplasmic mitochondrial DNA 3310 mutation in NADH dehydrogenase subunit 1 associated with type 2 diabetes, hypertrophic cardiomyopathy, and mental retardation in a single patient.
Diabetes Mellitus, Type 2
Insulin-regulated mitochondrial gene expression is associated with glucose flux in human skeletal muscle.
Diabetes Mellitus, Type 2
Loss of mTORC1 signalling impairs ?-cell homeostasis and insulin processing.
Diabetes Mellitus, Type 2
Metformin-treated patients with type 2 diabetes have normal mitochondrial complex I respiration.
Diabetes Mellitus, Type 2
Mitochondrial complex I activity is significantly decreased in a patient with maternally inherited type 2 diabetes mellitus and hypertrophic cardiomyopathy associated with mitochondrial DNA C3310T mutation: a cybrid study.
Diabetes Mellitus, Type 2
Mitochondrial DNA 3394 mutation in the NADH dehydrogenase subunit 1 associated with non-insulin-dependent diabetes mellitus.
Diabetes Mellitus, Type 2
Pancreatic mitochondrial complex I exhibits aberrant hyperactivity in diabetes.
Diabetes Mellitus, Type 2
Patients with type 2 diabetes have normal mitochondrial function in skeletal muscle.
Diabetes Mellitus, Type 2
Phosphorylation of PRAS40 on Thr246 by PKB/AKT facilitates efficient phosphorylation of Ser183 by mTORC1.
Diabetes Mellitus, Type 2
The GID ubiquitin ligase complex is a regulator of AMPK activity and organismal lifespan.
Diabetes Mellitus, Type 2
The prevalence of mitochondrial gene mutations in childhood diabetes in Japan.
Diabetes Mellitus, Type 2
The ROS production induced by a reverse-electron flux at respiratory-chain complex 1 is hampered by metformin.
Diabetes Mellitus, Type 2
TOM40 Inhibits Ovarian Cancer Cell Growth by Modulating Mitochondrial Function Including Intracellular ATP and ROS Levels.
Diabetes Mellitus, Type 2
Whole-blood transcriptome profiling reveals signatures of metformin and its therapeutic response.
Diabetic Nephropathies
Deficiency in apoptosis inducing factor recapitulates chronic kidney disease via aberrant mitochondrial homeostasis.
Diabetic Nephropathies
Role of Ragulator in the Regulation of Mechanistic Target of Rapamycin Signaling in Podocytes and Glomerular Function.
Diabetic Nephropathies
[Regulative mechanisms of mammalian target of rapamycin signaling pathway in glomerular hypertrophy in diabetic nephropathy and interventional effects of Chinese herbal medicine].
Diffuse Cerebral Sclerosis of Schilder
Dissecting the neuronal vulnerability underpinning Alpers' syndrome: a clinical and neuropathological study.
Down Syndrome
Decreased protein levels of complex I 30-kDa subunit in fetal Down syndrome brains.
Down Syndrome
Epigallocatechin-3-Gallate Plus Omega-3 Restores the Mitochondrial Complex I and F0F1-ATP Synthase Activities in PBMCs of Young Children with Down Syndrome: A Pilot Study of Safety and Efficacy.
Down Syndrome
Methylation of p15 and p16 genes in adult acute leukemia: lack of prognostic significance.
Down Syndrome
The reduction of NADH ubiquinone oxidoreductase 24- and 75-kDa subunits in brains of patients with Down syndrome and Alzheimer's disease.
Drug Resistant Epilepsy
Convergent and Divergent Mechanisms of Epileptogenesis in mTORopathies.
Dyskeratosis Congenita
Effects of protein extract from head-foot tissue of Oncomelania hupensis on the growth and gene expression of mother sporocysts of Schistosoma japonicum.
Dyskinesias
Dopamine- and cAMP-regulated Phosphoprotein of 32-kDa (DARPP-32)-dependent Activation of Extracellular Signal-regulated Kinase (ERK) and Mammalian Target of Rapamycin Complex 1 (mTORC1) Signaling in Experimental Parkinsonism.
Dyskinesias
L-DOPA-Induced Dyskinesia and Abnormal Signaling in Striatal Medium Spiny Neurons: Focus on Dopamine D1 Receptor-Mediated Transmission.
Dyskinesias
mTORC1 signaling in Parkinson's disease and L-DOPA-induced dyskinesia: A sensitized matter.
Dyskinesias
Mutation in NDUFA13/GRIM19 leads to early onset hypotonia, dyskinesia and sensorial deficiencies, and mitochondrial complex I instability.
Dyslipidemias
NADH dehydrogenase subunit-2 237 Leu/Met polymorphism modifies effects of cigarette smoking on risk of elevated levels of serum liver enzyme in male Japanese health check-up examinees: a cross-sectional study.
Dyslipidemias
PRAS40 suppresses atherogenesis through inhibition of mTORC1-dependent pro-inflammatory signaling in endothelial cells.
Dystonia
A frameshift mitochondrial complex I gene mutation in a patient with dystonia and cataracts: is the mutation pathogenic?
Dystonia
A mitochondrial DNA mutation at nucleotide pair 14459 of the NADH dehydrogenase subunit 6 gene associated with maternally inherited Leber hereditary optic neuropathy and dystonia.
Dystonia
A novel ND3 mitochondrial DNA mutation in three Korean children with basal ganglia lesions and complex I deficiency.
Dystonia
A novel recurrent mitochondrial DNA mutation in ND3 gene is associated with isolated complex I deficiency causing Leigh syndrome and dystonia.
Dystonia
Biochemical and genetic analysis of 3-methylglutaconic aciduria type IV: a diagnostic strategy.
Dystonia
Complex I respiratory defect in LHON plus dystonia with no mitochondrial DNA mutation.
Dystonia
Genetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystonia.
Dystonia
Leigh disease caused by the mitochondrial DNA G14459A mutation in unrelated families.
Dystonia
Mitochondrial complex I NUBPL mutations cause combined dystonia with bilateral striatal necrosis and cerebellar atrophy.
Dystonia
Mutations in the mitochondrial complex I assembly factor NDUFAF6 cause isolated bilateral striatal necrosis and progressive dystonia in childhood.
Dystonia
Progressive myoclonic epilepsy as an adult-onset manifestation of Leigh syndrome due to m.14487T>C.
Dystonia
Respiratory chain and mitochondrial deoxyribonucleic acid in blood cells from patients with focal and generalized dystonia.
Dystonia
Use of transmitochondrial cybrids to assign a complex I defect to the mitochondrial DNA-encoded NADH dehydrogenase subunit 6 gene mutation at nucleotide pair 14459 that causes Leber hereditary optic neuropathy and dystonia.
Dystonia
[A patient with bilateral lesion in the striatum and slowly progressive dystonia secondary to T14487C mutation in the ND6 gene of complex I of the mitochondrial respiratory chain]
Dystonic Disorders
A heteroplasmic mitochondrial complex I gene mutation in adult-onset dystonia.
Echinococcosis
Development of Three PCR Assays for the Differentiation between Echinococcus shiquicus, E. granulosus (G1 genotype), and E. multilocularis DNA in the Co-Endemic Region of Qinghai-Tibet plateau, China.
Echinococcosis
Genetic characterization of human-derived hydatid cysts of Echinococcus granulosus sensu lato in Heilongjiang Province and the first report of G7 genotype of E. canadensis in humans in China.
Echinococcosis
Genetic characterization of human-derived hydatid fluid based on mitochondrial gene sequencing in individuals from northern and western China.
Echinococcosis
Molecular Genotyping of the Human Cystic Echinococcosis in Mazandaran Province, North of Iran.
Echinococcosis
Sequence analysis of cox1 and nad1 genes in Echinococcus granulosus G3 genotype in camels (Camelus dromedarius) from central Iran.
Echinococcosis
The first report of human-derived G10 genotype of Echinococcus canadensis in China and possible sources and routes of transmission.
Encephalomyelitis
Gene Therapy with Single-Subunit Yeast NADH-Ubiquinone Oxidoreductase (NDI1) Improves the Visual Function in Experimental Autoimmune Encephalomyelitis (EAE) Mice Model of Multiple Sclerosis (MS).
Encephalomyelitis
GRIM-19 Ameliorates Multiple Sclerosis in a Mouse Model of Experimental Autoimmune Encephalomyelitis with Reciprocal Regulation of IFN?/Th1 and IL-17A/Th17 Cells.
Encephalomyelitis
NADH-dehydrogenase Type-2 Suppresses Irreversible Visual Loss and Neurodegeneration in the EAE Animal Model of MS.
Encephalomyelitis
The potential effects of hemp seed/evening primrose oils on the mammalian target of rapamycin complex 1 and interferon-gamma genes expression in experimental autoimmune encephalomyelitis.
Encephalomyelitis, Autoimmune, Experimental
Gene Therapy with Single-Subunit Yeast NADH-Ubiquinone Oxidoreductase (NDI1) Improves the Visual Function in Experimental Autoimmune Encephalomyelitis (EAE) Mice Model of Multiple Sclerosis (MS).
Encephalomyelitis, Autoimmune, Experimental
GRIM-19 Ameliorates Multiple Sclerosis in a Mouse Model of Experimental Autoimmune Encephalomyelitis with Reciprocal Regulation of IFN?/Th1 and IL-17A/Th17 Cells.
Encephalomyelitis, Autoimmune, Experimental
NADH-dehydrogenase Type-2 Suppresses Irreversible Visual Loss and Neurodegeneration in the EAE Animal Model of MS.
Encephalomyelitis, Autoimmune, Experimental
The potential effects of hemp seed/evening primrose oils on the mammalian target of rapamycin complex 1 and interferon-gamma genes expression in experimental autoimmune encephalomyelitis.
Endometrial Hyperplasia
Mitochondrial Dysfunctions in Type I Endometrial Carcinoma: Exploring Their Role in Oncogenesis and Tumor Progression.
Endometrial Neoplasms
Dual mTORC1/2 inhibition in a preclinical xenograft tumor model of endometrial cancer.
Endometrial Neoplasms
Increase in proteins involved in mitochondrial fission, mitophagy, proteolysis and antioxidant response in type I endometrial cancer as an adaptive response to respiratory complex I deficiency.
Endometrial Neoplasms
Mammalian target of rapamycin complex 1 and cyclooxygenase 2 pathways cooperatively exacerbate endometrial cancer.
Endometrial Neoplasms
mTOR-Dependent Role of Sestrin2 in Regulating Tumor Progression of Human Endometrial Cancer.
Endometrial Neoplasms
mTORC1 inhibitors: is temsirolimus in renal cancer telling us how they really work?
Endometrial Neoplasms
Obesity is associated with higher 4E-BP1 expression in endometrial cancer.
Endometrial Neoplasms
Stromal Liver Kinase B1 [STK11] Signaling Loss Induces Oviductal Adenomas and Endometrial Cancer by Activating Mammalian Target of Rapamycin Complex 1.
Endometriosis
Mitochondrial NADH:ubiquinone oxidoreductase alterations are associated with endometriosis.
Ependymoma
Nucleoporin TPR (translocated promoter region, nuclear basket protein) upregulation alters MTOR-HSF1 trails and suppresses autophagy induction in ependymoma.
Epilepsia Partialis Continua
Clinical, biochemical, and genetic features associated with VARS2-related mitochondrial disease.
Epilepsia Partialis Continua
Epilepsia partialis continua associated with NADH-coenzyme Q reductase deficiency.
Epilepsies, Myoclonic
An autopsy case of mitochondrial encephalomyopathy with prominent degeneration in olivo-ponto-cerebellar system.
Epilepsies, Myoclonic
Mutant NDUFV1 subunit of mitochondrial complex I causes leukodystrophy and myoclonic epilepsy.
Epilepsies, Partial
A human minisatellite hosts an alternative transcription start site for NPRL3 driving its expression in a repeat number-dependent manner.
Epilepsy
A homozygous variant in NDUFA8 is associated with developmental delay, microcephaly, and epilepsy due to mitochondrial complex I deficiency.
Epilepsy
A mouse model of DEPDC5-related epilepsy: Neuronal loss of Depdc5 causes dysplastic and ectopic neurons, increased mTOR signaling, and seizure susceptibility.
Epilepsy
Altered mitochondrial acetylation profiles in a kainic acid model of temporal lobe epilepsy.
Epilepsy
Bosch-Boonstra-Schaaf Optic Atrophy Syndrome Presenting as New-Onset Psychosis in a 32-Year-Old Man: A Case Report and Literature Review.
Epilepsy
Commentary on "Bosch-Boonstra-Schaaf Optic Atrophy Syndrome Presenting as New-Onset Psychosis in a 32-Year-Old Man: A Case Report and Literature Review".
Epilepsy
Control of Granule Cell Dispersion by Natural Materials Such as Eugenol and Naringin: A Potential Therapeutic Strategy Against Temporal Lobe Epilepsy.
Epilepsy
De novo and bi-allelic variants in AP1G1 cause neurodevelopmental disorder with developmental delay, intellectual disability, and epilepsy.
Epilepsy
Epilepsy and respiratory chain defects in children with mitochondrial encephalopathies.
Epilepsy
Immunohistochemical analysis of the oxidative phosphorylation complexes in skeletal muscle from patients with mitochondrial DNA encoded tRNA gene defects.
Epilepsy
Inhibition of the mammalian target of rapamycin blocks epilepsy progression in NS-Pten conditional knockout mice.
Epilepsy
KICSTOR recruits GATOR1 to the lysosome and is necessary for nutrients to regulate mTORC1.
Epilepsy
Mitochondrial complex I deficiency in the epileptic focus of patients with temporal lobe epilepsy.
Epilepsy
Pharmacological intervention to restore connectivity deficits of neuronal networks derived from ASD patient iPSC with a TSC2 mutation.
Epilepsy
Phytocannabinoid-dependent mTORC1 regulation is dependent upon inositol polyphosphate multikinase activity.
Epilepsy
PI3K-mTOR-S6K Signaling Mediates Neuronal Viability via Collapsin Response Mediator Protein-2 Expression.
Epilepsy
Progressive myoclonic epilepsy due to rare mitochondrial ND6 mutation, m.14487T>C.
Epilepsy
Rapamycin attenuates the development of posttraumatic epilepsy in a mouse model of traumatic brain injury.
Epilepsy
Regulable neural progenitor-specific Tsc1 loss yields giant cells with organellar dysfunction in a model of tuberous sclerosis complex.
Epilepsy
Selective inhibition of mTORC1/2 or PI3K/mTORC1/2 signaling does not prevent or modify epilepsy in the intrahippocampal kainate mouse model.
Epilepsy
Targeting deficiencies in mitochondrial respiratory complex I and functional uncoupling exerts anti-seizure effects in a genetic model of temporal lobe epilepsy and in a model of acute temporal lobe seizures.
Epilepsy, Temporal Lobe
Mechanistic target of rapamycin complex 1 and 2 in human temporal lobe epilepsy.
Epilepsy, Temporal Lobe
Mitochondrial complex I deficiency in the epileptic focus of patients with temporal lobe epilepsy.
Epilepsy, Temporal Lobe
Targeting deficiencies in mitochondrial respiratory complex I and functional uncoupling exerts anti-seizure effects in a genetic model of temporal lobe epilepsy and in a model of acute temporal lobe seizures.
Esophageal Motility Disorders
Endotracheal Intubation Using Alfentanil in a Pediatric Patient with a Mitochondrial Myopathy and Gastroparesis.
Esophageal Neoplasms
Bile acid exposure up-regulates tuberous sclerosis complex 1/mammalian target of rapamycin pathway in Barrett's-associated esophageal adenocarcinoma.
Esophageal Neoplasms
High incidence of coding gene mutations in mitochondrial DNA in esophageal cancer.
Esophageal Neoplasms
MicroRNA-141-3p regulates cellular proliferation, migration, and invasion in esophageal cancer by targeting tuberous sclerosis complex 1.
Esophageal Neoplasms
Synthesis, crystal structure and biological activity of two Mn complexes with 4-acyl pyrazolone derivatives.
Essential Hypertension
Interaction between mitochondrial NADH dehydrogenase subunit-2 5178 C > A and clinical risk factors on the susceptibility of essential hypertension in Chinese population.
Eye Diseases
Apoptosis inducing factor deficiency causes retinal photoreceptor degeneration. The protective role of the redox compound methylene blue.
Fanconi Anemia
Mitochondrial respiratory chain Complex I defects in Fanconi anemia complementation group A.
Fanconi Syndrome
Acadian variant of Fanconi syndrome is caused by mitochondrial respiratory chain complex I deficiency due to a non-coding mutation in complex I assembly factor NDUFAF6.
Fanconi Syndrome
Evolution, structure and membrane association of NDUFAF6, an assembly factor for NADH:ubiquinone oxidoreductase (Complex I).
Fatty Liver
Berberine alleviates lipid metabolism disorders via inhibition of mitochondrial complex I in gut and liver.
Fatty Liver
Betulin attenuated liver damage by prevention of hepatic mitochondrial dysfunction in rats with alcoholic steatohepatitis.
Fatty Liver
Effects of rosiglitazone on the liver histology and mitochondrial function in ob/ob mice.
Fatty Liver
Glutaminolysis-induced mTORC1 activation drives non-alcoholic steatohepatitis progression.
Fatty Liver
Highly expressed MT-ND3 positively associated with histological severity of hepatic steatosis.
Fatty Liver
Imeglimin Normalizes Glucose Tolerance and Insulin Sensitivity and Improves Mitochondrial Function in Liver of a High-Fat High-Sucrose Diet Mice Model.
Fatty Liver
Molecular mechanisms related to the hepatoprotective effects of antioxidant-rich extra virgin olive oil supplementation in rats subjected to short-term iron administration.
Fetal Death
Heightened uterine mammalian target of rapamycin complex 1 (mTORC1) signaling provokes preterm birth in mice.
Fetal Growth Retardation
Lower oxygen consumption and Complex I activity in mitochondria isolated from skeletal muscle of fetal sheep with intrauterine growth restriction.
Fetal Growth Retardation
Mechanistic Target of Rapamycin Complex 1 Promotes the Expression of Genes Encoding Electron Transport Chain Proteins and Stimulates Oxidative Phosphorylation in Primary Human Trophoblast Cells by Regulating Mitochondrial Biogenesis.
Fetal Growth Retardation
Prenatal Mechanistic Target of Rapamycin Complex 1 (m TORC1) Inhibition by Rapamycin Treatment of Pregnant Mice Causes Intrauterine Growth Restriction and Alters Postnatal Cardiac Growth, Morphology, and Function.
Fetal Growth Retardation
Severe congenital lactic acidosis and hypertrophic cardiomyopathy caused by an intronic variant in NDUFB7.
Fibroadenoma
Differential expression of the mitochondrial gene cytochrome oxidase II in benign and malignant breast tissue.
Fibroma
Periungual fibroma (Koenen tumors) as isolated sign of tuberous sclerosis complex with tuberous sclerosis complex 1 germline mutation.
Fragile X Syndrome
Dysregulation of Mammalian Target of Rapamycin Signaling in Mouse Models of Autism.
Friedreich Ataxia
Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease.
Friedreich Ataxia
Complex I and ATP content deficiency in lymphocytes from Friedreich's ataxia.
Friedreich Ataxia
Gene Expression Profiling of Mitochondrial Oxidative Phosphorylation (OXPHOS) Complex I in Friedreich Ataxia (FRDA) Patients.
Frontotemporal Dementia
Annonacin, a natural lipophilic mitochondrial complex I inhibitor, increases phosphorylation of tau in the brain of FTDP-17 transgenic mice.
Frontotemporal Dementia
C9orf72 ALS-FTD: recent evidence for dysregulation of the autophagy-lysosome pathway at multiple levels.
Frontotemporal Dementia
C9orf72 and smcr8 mutant mice reveal MTORC1 activation due to impaired lysosomal degradation and exocytosis.
Frontotemporal Dementia
SETX (senataxin), the helicase mutated in AOA2 and ALS4, functions in autophagy regulation.
Frontotemporal Lobar Degeneration
Genetic Variation of MT-ND Genes in Frontotemporal Lobar Degeneration: Biochemical Phenotype-Genotype Correlation.
Gait Ataxia
Late-Onset Leigh Syndrome due to NDUFV1 Mutation in a 10-Year-Old Boy Initially Presenting with Ataxia.
Gallbladder Neoplasms
Cordycepin down-regulates multiple drug resistant (MDR)/HIF-1? through regulating AMPK/mTORC1 signaling in GBC-SD gallbladder cancer cells.
Gastritis, Atrophic
Atrophic gastritis: deficient complex I of the respiratory chain in the mitochondria of corpus mucosal cells.
Gastritis, Atrophic
No evidence of a role for mitochondrial complex I in Helicobacter pylori pathogenesis.
Gastroparesis
Endotracheal Intubation Using Alfentanil in a Pediatric Patient with a Mitochondrial Myopathy and Gastroparesis.
Genetic Diseases, Inborn
Coding and small non-coding transcriptional landscape of tuberous sclerosis complex cortical tubers: implications for pathophysiology and treatment.
Genetic Diseases, Inborn
Dysfunction of mitochondrial respiratory chain complex I in neurological disorders: genetics and pathogenetic mechanisms.
Genetic Diseases, Inborn
Glutamate Stimulation Dysregulates AMPA Receptors-Induced Signal Transduction Pathway in Leber's Inherited Optic Neuropathy Patient-Specific hiPSC-Derived Retinal Ganglion Cells.
Genetic Diseases, Inborn
Leber hereditary optic neuropathy: involvement of the mitochondrial ND1 gene and evidence for an intragenic suppressor mutation.
Genetic Diseases, Inborn
Loss of Tsc1, but not Pten, in renal tubular cells causes polycystic kidney disease by activating mTORC1.
Genetic Diseases, Inborn
MicroRNA-34a activation in tuberous sclerosis complex during early brain development may lead to impaired corticogenesis.
Genetic Diseases, Inborn
Mitochondrial transport mediates survival of retinal ganglion cells in affected LHON patients.
Genetic Diseases, Inborn
Pathogenetic mechanisms in hereditary dysfunctions of complex I of the respiratory chain in neurological diseases.
Genetic Diseases, Inborn
Prolonging the survival of Tsc2 conditional knockout mice by glutamine supplementation.
Genetic Diseases, Inborn
Protective Role of rAAV-NDI1, Serotype 5, in an Acute MPTP Mouse Parkinson's Model.
Genetic Diseases, Inborn
Review of the treatment options for epilepsy in tuberous sclerosis complex: towards precision medicine.
Genetic Diseases, Inborn
Shotgun proteomics for the characterization of subunit composition of mitochondrial complex I.
Genetic Diseases, Inborn
TFEB activation restores migration ability to Tsc1-deficient adult neural stem/progenitor cells.
Genetic Diseases, Inborn
The Characterization of a Subependymal Giant Astrocytoma-Like Cell Line from Murine Astrocyte with mTORC1 Hyperactivation.
Genetic Diseases, Inborn
Tuberous sclerosis complex, mTOR, and the kidney: report of an NIDDK-sponsored workshop.
Genetic Diseases, Inborn
Vps34-mediated macropinocytosis in Tuberous Sclerosis Complex 2-deficient cells supports tumorigenesis.
Glaucoma
A cell-permeable phosphine-borane complex delays retinal ganglion cell death after axonal injury through activation of the pro-survival extracellular signal-regulated kinases 1/2 pathway.
Glaucoma
Rescue of cell death and inflammation of a mouse model of complex 1-mediated vision loss by repurposed drug molecules.
Glaucoma
Whole-mitochondrial genome sequencing in primary open-angle glaucoma using massively parallel sequencing identifies novel and known pathogenic variants.
Glaucoma, Open-Angle
Rescue of cell death and inflammation of a mouse model of complex 1-mediated vision loss by repurposed drug molecules.
Glaucoma, Open-Angle
Whole-mitochondrial genome sequencing in primary open-angle glaucoma using massively parallel sequencing identifies novel and known pathogenic variants.
Glioblastoma
Combinatorial Therapeutic Effect of Inhibitors of Aldehyde Dehydrogenase and Mitochondrial Complex I, and the Chemotherapeutic Drug, Temozolomide against Glioblastoma Tumorspheres.
Glioblastoma
Complex I inhibition augments dichloroacetate cytotoxicity through enhancing oxidative stress in VM-M3 glioblastoma cells.
Glioblastoma
DDR1 (discoidin domain receptor tyrosine kinase 1) drives glioblastoma therapy resistance by modulating autophagy.
Glioblastoma
GRIM-19 opposes reprogramming of glioblastoma cell metabolism via HIF1? destabilization.
Glioblastoma
Loss of Tsc1 accelerates malignant gliomagenesis when combined with oncogenic signals.
Glioblastoma
Mammalian target of rapamycin complex 1 activation sensitizes human glioma cells to hypoxia-induced cell death.
Glioblastoma
Metabolic-targeted Combination Therapy With Dichloroacetate and Metformin Suppresses Glioblastoma Cell Line Growth In Vitro and In Vivo.
Glioblastoma
Regulation of bioenergetics through dual inhibition of aldehyde dehydrogenase and mitochondrial complex I suppresses glioblastoma tumorspheres.
Glioblastoma
Second Generation mTOR Inhibitors as a Double-Edged Sword in Malignant Glioma Treatment.
Glioblastoma
The cytotoxicity of gallium maltolate in glioblastoma cells is enhanced by metformin through combined action on mitochondrial complex 1.
Glioblastoma
The physiological mTOR complex 1 inhibitor DDIT4 mediates therapy resistance in glioblastoma.
Glioma
5-Hydroxy-1,4-naphthalenedione exerts anticancer effects on glioma cells through interaction with the mitochondrial electron transport chain.
Glioma
Adaptor Protein Complex 1 Sigma 3 Is Highly Expressed in Glioma and Could Enhance Its Progression.
Glioma
Contribution of the phosphorylable complex I in the growth phase-dependent respiration of C6 glioma cells in vitro.
Glioma
Dinuclear Pt(ii)-bisphosphonate complexes: a scaffold for multinuclear or different oxidation state platinum drugs.
Glioma
Immunohistochemical Assessment of Phosphorylated mTORC1-Pathway Proteins in Human Brain Tumors.
Glioma
Increased efficacy of an interleukin-12-secreting herpes simplex virus in a syngeneic intracranial murine glioma model.
Glioma
Inhibition of mitochondria NADH-Ubiquinone oxidoreductase (complex I) sensitizes the radioresistant glioma U87MG cells to radiation.
Glioma
Loss of Tsc1 accelerates malignant gliomagenesis when combined with oncogenic signals.
Glioma
Mammalian target of rapamycin complex 1 activation sensitizes human glioma cells to hypoxia-induced cell death.
Glioma
MicroRNA-351 Promotes the Proliferation and Invasion of Glioma Cells through Downregulation of NAIF1.
Glioma
Mutation in mitochondrial complex I ND6 subunit is associated with defective response to hypoxia in human glioma cells.
Glioma
Repurposing phenformin for the targeting of glioma stem cells and the treatment of glioblastoma.
Glioma
Tafazzin-dependent cardiolipin composition in C6 glioma cells correlates with changes in mitochondrial and cellular functions, and cellular proliferation.
Glioma
Therapeutic strategy for targeting aggressive malignant gliomas by disrupting their energy balance.
Glucose Intolerance
IRS1Ser³?? phosphorylation does not mediate mTORC1-induced insulin resistance.
Glucose Intolerance
Loss of TSC complex enhances gluconeogenesis via upregulation of Dlk1-Dio3 locus miRNAs.
glycogen phosphorylase deficiency
Myophosphorylase deficiency associated with a defect in complex I of the mitochondrial respiratory chain.
Glycogen Storage Disease
Quantitative analysis of proteins of metabolism by reverse phase protein microarrays identifies potential biomarkers of rare neuromuscular diseases.
Glycogen Storage Disease
Whole-exome Sequencing Helps the Diagnosis and Treatment in Children with Neurodevelopmental Delay Accompanied Unexplained Dyspnea.
Glycogen Storage Disease Type V
Myophosphorylase deficiency associated with a defect in complex I of the mitochondrial respiratory chain.
Gout
[Ru(bpy)2(NO)SO3](PF6), a Nitric Oxide Donating Ruthenium Complex, Reduces Gout Arthritis in Mice.
Granuloma
High expression of mTOR signaling in granulomatous lesions is not predictive for the clinical course of sarcoidosis.
h+-transporting two-sector atpase deficiency
Noninvasive diagnostics of mitochondrial disorders in isolated lymphocytes with high resolution respirometry.
Hamartoma
Human TSC2-null fibroblast-like cells induce hair follicle neogenesis and hamartoma morphogenesis.
Hamartoma
Unrestrained mammalian target of rapamycin complexes 1 and 2 increase expression of phosphatase and tensin homolog deleted on chromosome 10 to regulate phosphorylation of Akt kinase.
Hamartoma Syndrome, Multiple
Familial non-medullary thyroid carcinoma: clinico-pathological features, current knowledge and novelty regarding genetic risk factors.
Head and Neck Neoplasms
A phase 1 study of everolimus plus docetaxel plus cisplatin as induction chemotherapy for patients with locally and/or regionally advanced head and neck cancer.
Hearing Loss
A novel AIFM1 missense mutation in a Japanese patient with ataxic sensory neuronopathy and hearing impairment.
Hearing Loss
Cerebellar ataxia, neuropathy, hearing loss, and intellectual disability due to AIFM1 mutation.
Hearing Loss
Gentamicin-induced hearing loss: A retrospective study using the Food and Drug Administration Adverse Event Reporting System and a toxicological study using drug-gene network analysis.
Hearing Loss
Mutations in AIFM1 cause an X-linked childhood cerebellar ataxia partially responsive to riboflavin.
Hearing Loss
X-linked hypomyelination with spondylometaphyseal dysplasia (H-SMD) associated with mutations in AIFM1.
Hearing Loss
[PROVISIONAL] Whole exome sequencing identifies a novel variant in an apoptosis-inducing factor gene associated with X-linked recessive hearing loss in a Chinese Family.
Hearing Loss, Bilateral
A novel m.11406 T > A mutation in mitochondrial ND4 gene causes MELAS syndrome.
Hearing Loss, Mixed Conductive-Sensorineural
The genotypic and phenotypic spectrum of PIGA deficiency.
Heart Arrest
A randomized and blinded trial of inhaled nitric oxide in a piglet model of pediatric cardiopulmonary resuscitation.
Heart Arrest
Early mitochondrial dysfunction in electron transfer activity and reactive oxygen species generation after cardiac arrest.
Heart Arrest
Nitric oxide attenuates cardiomyocytic apoptosis via diminished mitochondrial complex I up-regulation from cardiac ischemia-reperfusion injury under cardiopulmonary bypass.
Heart Arrest
Nitrite therapy after cardiac arrest reduces reactive oxygen species generation, improves cardiac and neurological function, and enhances survival via reversible inhibition of mitochondrial complex I.
Heart Arrest
Preservation of mitochondrial function with cardiopulmonary resuscitation in prolonged cardiac arrest in rats.
Heart Arrest
Suppression of Superoxide-Hydrogen Peroxide Production at Site IQ of Mitochondrial Complex I Attenuates Myocardial Stunning and Improves Postcardiac Arrest Outcomes.
Heart Block
Biventricular non-compaction hypertrophic cardiomyopathy in association with congenital complete heart block and type I mitochondrial complex deficiency.
Heart Diseases
Insulin upregulates GRIM-19 and protects cardiac mitochondrial morphology in type 1 diabetic rats partly through PI3K/AKT signaling pathway.
Heart Failure
Clinical impact of myocardial mTORC1 activation in nonischemic dilated cardiomyopathy.
Heart Failure
Differential effects of various genetic mouse models of the mechanistic target of rapamycin complex I inhibition on heart failure.
Heart Failure
Direct Cardiac Actions of the Sodium Glucose Co-Transporter 2 Inhibitor Empagliflozin Improve Myocardial Oxidative Phosphorylation and Attenuate Pressure-Overload Heart Failure.
Heart Failure
Mitochondrial complex I deficiency increases protein acetylation and accelerates heart failure.
Heart Failure
Mitochondrial respiratory control and early defects of oxidative phosphorylation in the failing human heart.
Heart Failure
Mitochondrials complex I activity is reduced in latent adriamycin-induced cardiomyopathy of rat.
Heart Failure
Pivotal Importance of STAT3 in Protecting the Heart from Acute and Chronic Stress: New Advancement and Unresolved Issues.
Heart Failure
Selective NADH communication from ?-ketoglutarate dehydrogenase to mitochondrial transhydrogenase prevents reactive oxygen species formation under reducing conditions in the heart.
Heart Failure
The role of reactive oxygen species in myocardial redox signaling and regulation.
Hematologic Diseases
BCOR-coupled H2A monoubiquitination represses a subset of androgen receptor target genes regulating prostate cancer proliferation.
Hematologic Neoplasms
Identification of novel fusion genes with 28S ribosomal DNA in hematologic malignancies.
Hemimegalencephaly
Contribution of Altered Endocannabinoid System to Overactive mTORC1 Signaling in Focal Cortical Dysplasia.
Hemolytic-Uremic Syndrome
Hemolytic uremic syndrome and rhabdomyolysis in a patient with succinate coenzyme Q reductase (complex II) deficiency.
Hepatic Infarction
Dietary nitrate and nitrite modulate blood and organ nitrite and the cellular ischemic stress response.
Hepatitis
GRIM-19 Restricts HCV Replication by Attenuating Intracellular Lipid Accumulation.
Hepatitis B
Glucosamine promotes hepatitis B virus replication through its dual effects in suppressing autophagic degradation and inhibiting MTORC1 signaling.
Hepatitis B
Prediction of transmembrane segments in proteins utilising multiple sequence alignments.
Hepatitis C
GRIM-19 Restricts HCV Replication by Attenuating Intracellular Lipid Accumulation.
Hepatitis C
HIV-1 Vpr protein impairs lysosome clearance causing SNCA/alpha-synuclein accumulation in neurons.
Hepatitis C, Chronic
Hepatitis C virus core protein inhibits mitochondrial electron transport and increases reactive oxygen species (ROS) production.
Hepatitis C, Chronic
Impaired interferon signaling in chronic hepatitis C patients with advanced fibrosis via the transforming growth factor beta signaling pathway.
Hermanski-Pudlak Syndrome
Hermansky-Pudlak syndrome type 7 (HPS-7) results from mutant dysbindin, a member of the biogenesis of lysosome-related organelles complex 1 (BLOC-1).
Herpes Simplex
Remodeling mTORC1 Responsiveness to Amino Acids by the Herpes Simplex Virus UL46 and Us3 Gene Products Supports Replication during Nutrient Insufficiency.
Herpes Zoster
Heterogeneous levels of oxidative phosphorylation enzymes in rat adrenal glands.
Herpes Zoster
Varicella Zoster Virus Downregulates Programmed Death Ligand 1 and Major Histocompatibility Complex 1 in Human Brain Vascular Adventitial Fibroblasts, Perineurial Cells and Lung Fibroblasts.
HIV Infections
Mitochondrial complex I activity is impaired during HIV-1-induced T-cell apoptosis.
Homocystinuria
Whole-exome Sequencing Helps the Diagnosis and Treatment in Children with Neurodevelopmental Delay Accompanied Unexplained Dyspnea.
Huntington Disease
A single-subunit NADH-quinone oxidoreductase renders resistance to mammalian nerve cells against complex I inhibition.
Huntington Disease
AMPK-dependent phosphorylation is required for transcriptional activation of TFEB and TFE3.
Huntington Disease
Buccal Respiratory Chain Complexes I and IV Quantities in Huntington's Disease Patients.
Huntington Disease
Evidence for a defect in NADH: ubiquinone oxidoreductase (complex I) in Huntington's disease.
Huntington Disease
Identification of genes associated with the effect of inflammation on the neurotransmission of vascular smooth muscle cell.
Huntington Disease
Isolated cerebral and cerebellar mitochondria produce free radicals when exposed to elevated CA2+ and Na+: implications for neurodegeneration.
Huntington Disease
Mitochondrial cyclic AMP response element-binding protein (CREB) mediates mitochondrial gene expression and neuronal survival.
Huntington Disease
Mitochondrial Respiratory Function in Peripheral Blood Cells from Huntington's Disease Patients.
Huntington Disease
Mutant HTT (huntingtin) impairs mitophagy in a cellular model of Huntington disease.
Huntington Disease
Normal platelet mitochondrial complex I activity in Huntington's disease.
Huntington Disease
NR2B-containing NMDA receptors promote the neurotoxic effects of 3-nitropropionic acid but not of rotenone in the striatum.
Huntington Disease
Quantitative autoradiography of dihydrorotenone binding to complex I of the electron transport chain.
Hydrocephalus
Congenital ocular motor apraxia associated with myopathy, external hydrocephalus and NADH dehydrogenase deficiency.
Hyperalgesia
Increased production of mitochondrial superoxide in the spinal cord induces pain behaviors in mice: the effect of mitochondrial electron transport complex inhibitors.
Hyperalgesia
Intact subepidermal nerve fibers mediate mechanical hypersensitivity via the activation of protein kinase C gamma in spared nerve injury.
Hyperalgesia
Intrathecal rapamycin attenuates morphine-induced analgesic tolerance and hyperalgesia in rats with neuropathic pain.
Hyperalgesia
mTOR, a new potential target for chronic pain and opioid-induced tolerance and hyperalgesia.
Hyperalgesia
Systemic Rapamycin Attenuates Morphine-Induced Analgesic Tolerance and Hyperalgesia in Mice.
Hyperalgesia
The ruthenium NO donor, [Ru(bpy)2(NO)SO3](PF6), inhibits inflammatory pain: Involvement of TRPV1 and cGMP/PKG/ATP-sensitive potassium channel signaling pathway.
Hyperglycemia
Effects of Moringa oleifera Leaves Extract on High Glucose-Induced Metabolic Changes in HepG2 Cells.
Hyperglycemia
Functional deficiencies of subsarcolemmal mitochondria in the type 2 diabetic human heart.
Hyperglycemia
Hyperglycemia in a type 1 Diabetes Mellitus model causes a shift in mitochondria coupled-glucose phosphorylation and redox metabolism in rat brain.
Hyperglycemia
Metformin Improves Mitochondrial Respiratory Activity through Activation of AMPK.
Hyperglycemia
Metformin protects high glucose?cultured cardiomyocytes from oxidative stress by promoting NDUFA13 expression and mitochondrial biogenesis via the AMPK signaling pathway.
Hyperglycemia
Roles of Pyruvate, NADH, and Mitochondrial Complex I in Redox Balance and Imbalance in ? Cell Function and Dysfunction.
Hyperglycemia
Superoxide production by mitochondria of insulin-sensitive tissues: mechanistic differences and effect of early diabetes.
Hyperglycemia
Synergistic exacerbation of mitochondrial and synaptic dysfunction and resultant learning and memory deficit in a mouse model of diabetic Alzheimer's disease.
Hyperglycemia
Upregulation of the mTOR Complex 1 Pathway by Rheb in Pancreatic {beta} Cells Leads to Increased {beta} Cell Mass and Prevention of Hyperglycemia.
Hyperparathyroidism, Secondary
Phosphorylation of Ribosomal Protein S6 Mediates Mammalian Target of Rapamycin Complex 1-Induced Parathyroid Cell Proliferation in Secondary Hyperparathyroidism.
Hypersensitivity
Altered anesthetic sensitivity of mice lacking ndufs4, a subunit of mitochondrial complex I.
Hypersensitivity
Isoflurane anesthetic hypersensitivity and progressive respiratory depression in a mouse model with isolated mitochondrial complex I deficiency.
Hypersensitivity
Isoflurane Selectively Inhibits Distal Mitochondrial Complex I in Caenorhabditis Elegans.
Hypersensitivity
Mitochondrial Function in Astrocytes Is Essential for Normal Emergence from Anesthesia in Mice.
Hypersensitivity
The emerging role of autophagic-lysosomal dysfunction in Gaucher disease and Parkinson's disease.
Hypersensitivity
[Ru(bpy)2(NO)SO3](PF6), a Nitric Oxide Donating Ruthenium Complex, Reduces Gout Arthritis in Mice.
Hypertension
A novel complex I inhibitor protects against hypertension-induced left ventricular hypertrophy.
Hypertension
Comparative effects of avocado oil and losartan on blood pressure, renal vascular function, and mitochondrial oxidative stress in hypertensive rats.
Hypertension
Glutaredoxin-2 controls cardiac mitochondrial dynamics and energetics in mice, and protects against human cardiac pathologies.
Hypertension
Inhibition of Mammalian Target of Rapamycin Complex 1 Attenuates Salt-Induced Hypertension and Kidney Injury in Dahl Salt-Sensitive Rats.
Hypertension
Insufficient fumarase contributes to hypertension by an imbalance of redox metabolism in Dahl salt-sensitive rats.
Hypertension
Mitochondrial dysfunction in the hypertensive rat brain: respiratory complexes exhibit assembly defects in hypertension.
Hypertension
NADH dehydrogenase subunit-2 237 Leu/Met polymorphism modifies the effects of alcohol consumption on risk for hypertension in middle-aged Japanese men.
Hypertension
NADH dehydrogenase subunit-2 237 Leu/Met polymorphism modulates the effects of coffee consumption on the risk of hypertension in middle-aged Japanese men.
Hypertension
Skeletal muscle mitochondrial depletion and dysfunction in chronic kidney disease.
Hypertension
Up-regulation of the mammalian target of rapamycin complex 1 subunit Raptor by aldosterone induces abnormal pulmonary artery smooth muscle cell survival patterns to promote pulmonary arterial hypertension.
Hypertension
[Expression of nicotinamide edenine dinucleotide dehydrogenase gene in placenta of patients with pregnancy induced hypertension]
Hypertension, Pregnancy-Induced
[Expression of nicotinamide edenine dinucleotide dehydrogenase gene in placenta of patients with pregnancy induced hypertension]
Hypertension, Pulmonary
Congenital lactic acidosis, cerebral cysts and pulmonary hypertension in an infant with FOXRED1 related complex 1 deficiency.
Hypertension, Pulmonary
Congenital lactic acidosis, cerebral cysts and pulmonary hypertension in an infant with FOXRED1 related complex I deficiency.
Hypertension, Pulmonary
Roles of different mitochondrial electron transport chain complexes in hypoxia-induced pulmonary vasoconstriction.
Hypertension, Pulmonary
Selective Tuberous Sclerosis Complex 1 Gene Deletion in Smooth Muscle Activates Mammalian Target of Rapamycin Signaling and Induces Pulmonary Hypertension.
Hyperthyroidism
Alleviation of enhanced oxidative stress and oxygen consumption of L-thyroxine induced hyperthyroid rat liver mitochondria by vitamin E and curcumin.
Hyperthyroidism
Effects of hypo- and hyperthyroidism on the complex I activity in rat heart mitochondria.
Hyperthyroidism
On the thyroid hormone-induced increase in respiratory capacity of isolated rat hepatocytes.
Hypertrophy, Left Ventricular
A novel complex I inhibitor protects against hypertension-induced left ventricular hypertrophy.
Hypertrophy, Left Ventricular
Electron transport chain dysfunction in neonatal pressure-overload hypertrophy precedes cardiomyocyte apoptosis independent of oxidative stress.
Hypertrophy, Right Ventricular
Mitochondrial complex I dysfunction and altered NAD(P)H kinetics in rat myocardium in cardiac right ventricular hypertrophy and failure.
Hypertrophy, Right Ventricular
mTORC1 Is Involved in Hypoxia-Induced Pulmonary Hypertension Through the Activation of Notch3.
Hypoglycemia
Lower oxygen consumption and Complex I activity in mitochondria isolated from skeletal muscle of fetal sheep with intrauterine growth restriction.
Hypogonadism
Sporadic heteroplasmic single 5.5 kb mitochondrial DNA deletion associated with cerebellar ataxia, hypogonadotropic hypogonadism, choroidal dystrophy, and mitochondrial respiratory chain complex I deficiency.
Hypokinesia
Chronic systemic pesticide exposure reproduces features of Parkinson's disease.
Hypokinesia
Complex I syndrome in striatum and frontal cortex in a rat model of Parkinson disease.
Hypothyroidism
Effect of thyroid hormones on mitochondrial oxygen free radical production and DNA oxidative damage in the rat heart.
Hypothyroidism
Hypothyroidism decreases the biogenesis in free mitochondria and neuronal oxygen consumption in the cerebral cortex of developing rats.
Hypoxia-Ischemia, Brain
Redox-Dependent Loss of Flavin by Mitochondrial Complex I in Brain Ischemia/Reperfusion Injury.
Infections
?9-tetrahydrocannabinol impairs the inflammatory response to influenza infection: role of antigen-presenting cells and the cannabinoid receptors 1 and 2.
Infections
Acetaldehyde suppresses HBV-MHC class I complex presentation on hepatocytes via induction of ER stress and Golgi fragmentation.
Infections
Activities of membrane bound phosphatases, transaminases and mitochondrial enzymes in white spot syndrome virus infected tissues of Fenneropenaeus indicus.
Infections
Ancestral T Cells in Fish Require mTORC1-Coupled Immune Signals and Metabolic Programming for Proper Activation and Function.
Infections
Cellular proteins specifically bind single- and double-stranded DNA and RNA from the initiation site of a transcript that crosses the origin of DNA replication of herpes simplex virus 1.
Infections
Complex I binding by a virally encoded RNA regulates mitochondria-induced cell death.
Infections
Decreased expression of GRIM-19 and its association with high-risk HPV infection in cervical squamous intraepithelial neoplasias and cancer.
Infections
Dynein mediates the localization and activation of mTOR in normal and human cytomegalovirus-infected cells.
Infections
Effect of infectious bursal disease virus infection on energy metabolism in embryonic chicken livers.
Infections
Enigmatic presence of mitochondrial complex I in Trypanosoma brucei bloodstream forms.
Infections
Equine herpesvirus type 1 (EHV1) induces alterations in the immunophenotypic profile of equine monocyte-derived dendritic cells.
Infections
Function of GRIM-19, a Mitochondrial Respiratory Chain Complex I Protein, in Innate Immunity.
Infections
Genetic diversity and multiplicity of infection in Fasciola gigantica isolates of Pakistani livestock.
Infections
Genetic variation of Echinococcus spp. in yaks and sheep in the Tibet Autonomous Region of China based on mitochondrial DNA.
Infections
GRIM-19 Restricts HCV Replication by Attenuating Intracellular Lipid Accumulation.
Infections
Helicobacter pylori Infection Modulates Host Cell Metabolism through VacA-Dependent Inhibition of mTORC1.
Infections
Hepatocarcinogenesis in hepatitis C: HCV shrewdly exacerbates oxidative stress by modulating both production and scavenging of reactive oxygen species.
Infections
History of infection with different male-killing bacteria in the two-spot ladybird beetle Adalia bipunctata revealed through mitochondrial DNA sequence analysis.
Infections
HPV16E7 silencing enhances susceptibility of CaSki cells to natural killer cells.
Infections
IL-4 Protects the Mitochondria Against TNF? and IFN? Induced Insult During Clearance of Infection with Citrobacter rodentium and Escherichia coli.
Infections
In vitro and murine efficacy and toxicity studies of nebulized SCC1, a methylated caffeine-silver(I) complex, for treatment of pulmonary infections.
Infections
Induction of HOX Genes by Hepatitis C Virus Infection via Impairment of Histone H2A Monoubiquitination.
Infections
Infectious pancreatic necrosis virus enters CHSE-214 cells via macropinocytosis.
Infections
Intestinal Microbiota-Derived GABA Mediates Interleukin-17 Expression during Enterotoxigenic Escherichia coli Infection.
Infections
Intracellular forms of simian virus 40 nucleoprotein complexes. I. Methods of isolation and characterization in CV-1 cells.
Infections
Kaposi sarcoma-associated herpesvirus miRNAs suppress CASTOR1-mediated mTORC1 inhibition to promote tumorigenesis.
Infections
Manganese superoxide dismutase deficiency exacerbates the mitochondrial ROS production and oxidative damage in Chagas disease.
Infections
Mitochondrial GRIM-19 as a potential therapeutic target for STAT3-dependent carcinogenesis of gastric cancer.
Infections
Molecular cloning and multifunctional characterization of GRIM-19 (gene associated with retinoid-interferon-induced mortality 19) homologue from turbot (Scophthalmus maximus).
Infections
Molecular discrimination of Opisthorchis-like eggs from residents in a rural community of central Thailand.
Infections
Molecular genetic comparison of Onchocerca sp. infecting dogs in Europe with other spirurid nematodes including Onchocerca lienalis.
Infections
Mycoplasma pneumoniae infection induces reactive oxygen species and DNA damage in A549 human lung carcinoma cells.
Infections
Opsonophagocytosis-inhibiting mac protein of group a streptococcus: identification and characteristics of two genetic complexes.
Infections
Overexpression of apoptosis-inducing factor mitochondrion-associated 1 (AIFM1) induces apoptosis by promoting the transcription of caspase3 and DRAM in hepatoma cells.
Infections
Preliminary data on Ascaridia galli infections in Gallus gallus domesticus and the development of a specific primer based on the NADH dehydrogenase subunit 4.
Infections
Rabies virus phosphoprotein interacts with mitochondrial Complex I and induces mitochondrial dysfunction and oxidative stress.
Infections
Rationally designed curcumin based ruthenium(ii) antimicrobials effective against drug-resistant Staphylococcus aureus.
Infections
RNAi screening reveals requirement for host cell secretory pathway in infection by diverse families of negative-strand RNA viruses.
Infections
Serine residues at positions 162 and 166 of the rabies virus phosphoprotein are critical for the induction of oxidative stress in rabies virus infection.
Infections
Serp2, an inhibitor of the interleukin-1beta-converting enzyme, is critical in the pathobiology of myxoma virus.
Infections
The human cytomegalovirus non-coding Beta2.7 RNA as a novel therapeutic for Parkinson's disease - Translational research with no translation.
Infections
The U95 protein of human herpesvirus 6B interacts with human GRIM-19: silencing of U95 expression reduces viral load and abrogates loss of mitochondrial membrane potential.
Infections
TORC1 inhibition enhances immune function and reduces infections in the elderly.
Infections
Tuberous Sclerosis Complex Protein 2-Independent Activation of mTORC1 by Human Cytomegalovirus pUL38.
Infections
Type 2 NADH Dehydrogenase Is the Only Point of Entry for Electrons into the Streptococcus agalactiae Respiratory Chain and Is a Potential Drug Target.
Infections
Upregulation of human mitochondrial NADH dehydrogenase subunit 5 in intestinal epithelial cells is modulated by Vibrio cholerae pathogenesis.
Infections
Uropathogenic Escherichia coli Infection Compromises the Blood-Testis Barrier by Disturbing mTORC1-mTORC2 Balance.
Infections
[Effect of a number of preparations on the function of the immune system in experimental influenza in mice]
Infertility
Lack of association between single polymorphic variants of the mitochondrial nicotinamide adenine dinucleotide dehydrogenase 3, and 4L (MT-ND3 and MT-ND4L) and male infertility.
Infertility
Mitochondrial nicotinamide adenine dinucleotide hydride dehydrogenase (NADH) subunit 4 (MTND4) polymorphisms and their association with male infertility.
Infertility, Male
A NADH dehydrogenase subunit gene is co-transcribed with the abnormal Petunia mitochondrial gene associated with cytoplasmic male sterility.
Infertility, Male
DJ-1 deficiency causes metabolic abnormality in ornidazole-induced asthenozoospermia.
Infertility, Male
Identification of miRNAs and their targets by high-throughput sequencing and degradome analysis in cytoplasmic male-sterile line NJCMS1A and its maintainer NJCMS1B of soybean.
Infertility, Male
Mitochondrial gene organization and expression in petunia male fertile and sterile plants.
Infertility, Male
Mitochondrial nad2 gene is co-transcripted with CMS-associated orfB gene in cytoplasmic male-sterile stem mustard (Brassica juncea).
Infertility, Male
Organization and expression of the mitochondrial genome in the Nicotiana sylvestris CMSII mutant.
Infertility, Male
Transcription of the Petunia mitochondrial CMS-associated Pcf locus in male sterile and fertility-restored lines.
Inflammatory Bowel Diseases
GRIM-19 interacts with nucleotide oligomerization domain 2 and serves as downstream effector of anti-bacterial function in intestinal epithelial cells.
Inflammatory Bowel Diseases
Repression of Mammalian Target of Rapamycin Complex 1 Inhibits Intestinal Regeneration in Acute Inflammatory Bowel Disease Models.
Influenza, Human
?9-tetrahydrocannabinol impairs the inflammatory response to influenza infection: role of antigen-presenting cells and the cannabinoid receptors 1 and 2.
Influenza, Human
Comparative study of lymphocytes from individuals that were vaccinated and unvaccinated against the pandemic 2009-2011 H1N1 influenza virus in Southern Brazil.
Influenza, Human
Glucosamine promotes hepatitis B virus replication through its dual effects in suppressing autophagic degradation and inhibiting MTORC1 signaling.
Influenza, Human
Liver-specific mitochondrial respiratory chain complex I deficiency in fatal influenza encephalopathy.
Influenza, Human
Major histocompatibility complex I of swine respiratory cells presents conserved regions of influenza proteins.
Influenza, Human
p53 increases MHC class I expression by upregulating the endoplasmic reticulum aminopeptidase ERAP1.
Insulin Resistance
A pilot 'window of opportunity' neoadjuvant study of metformin in localised prostate cancer.
Insulin Resistance
A review of potential metabolic etiologies of the observed association between red meat consumption and development of type 2 diabetes mellitus.
Insulin Resistance
Activation of mammalian target of rapamycin complex 1 and insulin resistance induced by palmitate in hepatocytes.
Insulin Resistance
Acute treadmill exercise discriminately improves the skeletal muscle insulin-stimulated growth signaling responses in mice lacking REDD1.
Insulin Resistance
Adiponectin supplementation in pregnant mice prevents the adverse effects of maternal obesity on placental function and fetal growth.
Insulin Resistance
Aifm2, a NADH Oxidase, Supports Robust Glycolysis and Is Required for Cold- and Diet-Induced Thermogenesis.
Insulin Resistance
Altered branched chain amino acid metabolism: toward a unifying cardiometabolic hypothesis.
Insulin Resistance
Basal activation of p70S6K results in adipose-specific insulin resistance in protein-tyrosine phosphatase 1B -/- mice.
Insulin Resistance
Berberine and its more biologically available derivative, dihydroberberine, inhibit mitochondrial respiratory complex I: a mechanism for the action of berberine to activate AMP-activated protein kinase and improve insulin action.
Insulin Resistance
Cross-talks via mTORC2 can explain enhanced activation in response to insulin in diabetic patients.
Insulin Resistance
Differential localization and anabolic responsiveness of mTOR complexes in human skeletal muscle in response to feeding and exercise.
Insulin Resistance
Disruption of insulin receptor substrate-2 impairs growth but not insulin function in rats.
Insulin Resistance
Effects of a brief high-fat diet and acute exercise on the mTORC1 and IKK/NF-?B pathways in rat skeletal muscle.
Insulin Resistance
Enhanced skeletal muscle insulin sensitivity after acute resistance-type exercise is upregulated by rapamycin-sensitive mTOR complex 1 inhibition.
Insulin Resistance
Fibroblast Growth Factor 21 Improves Hepatic Insulin Sensitivity by Inhibiting Mammalian Target of Rapamycin Complex 1.
Insulin Resistance
Go-6976 reverses hyperglycemia-induced insulin resistance independently of cPKC inhibition in adipocytes.
Insulin Resistance
Htd2 deficiency-associated suppression of ?-lipoic acid production provokes mitochondrial dysfunction and insulin resistance in adipocytes.
Insulin Resistance
Increased reactive oxygen species production and lower abundance of complex I subunits and carnitine palmitoyltransferase 1B protein despite normal mitochondrial respiration in insulin-resistant human skeletal muscle.
Insulin Resistance
Inhibition of ERK1/2 Restores GSK3? Activity and Protein Synthesis Levels in a Model of Tuberous Sclerosis.
Insulin Resistance
Inhibition of mitochondrial complex 1 by the S6K1 inhibitor PF-4708671 partly contributes to its glucose metabolic effects in muscle and liver cells.
Insulin Resistance
Insulin Resistance without Elevated Mammalian Target of Rapamycin Complex 1 Activity in Muscles of Mice Fed a High Fat Diet.
Insulin Resistance
Insulin-regulated mitochondrial gene expression is associated with glucose flux in human skeletal muscle.
Insulin Resistance
Interplay between NADH oxidation by complex I, glutathione redox state and sirtuin-3, and its role in the development of insulin resistance.
Insulin Resistance
IRS1Ser³?? phosphorylation does not mediate mTORC1-induced insulin resistance.
Insulin Resistance
KLF15 is a molecular link between endoplasmic reticulum stress and insulin resistance.
Insulin Resistance
Liver damage, inflammation, and enhanced tumorigenesis after persistent mTORC1 inhibition.
Insulin Resistance
Mitochondrial complex I impairment in leukocytes from polycystic ovary syndrome patients with insulin resistance.
Insulin Resistance
Mitochondrial metabolism and type-2 diabetes: a specific target of metformin.
Insulin Resistance
NDUFAB1 protects against obesity and insulin resistance by enhancing mitochondrial metabolism.
Insulin Resistance
Novel nervous and multi-system regenerative therapeutic strategies for diabetes mellitus with mTOR.
Insulin Resistance
Pharmacological inhibition of S6K1 increases glucose metabolism and Akt signalling in vitro and in diet-induced obese mice.
Insulin Resistance
Pioglitazone leads to an inactivation and disassembly of complex I of the mitochondrial respiratory chain.
Insulin Resistance
Placental mTOR complex 1 regulates fetal programming of obesity and insulin resistance in mice.
Insulin Resistance
PRAS40 suppresses atherogenesis through inhibition of mTORC1-dependent pro-inflammatory signaling in endothelial cells.
Insulin Resistance
Puerarin attenuates palmitate-induced mitochondrial dysfunction, impaired mitophagy and inflammation in L6 myotubes.
Insulin Resistance
Rapamycin does not improve insulin sensitivity despite elevated mammalian target of rapamycin complex 1 activity in muscles of ob/ob mice.
Insulin Resistance
Rapamycin has a biphasic effect on insulin sensitivity in C2C12 myotubes due to sequential disruption of mTORC1 and mTORC2.
Insulin Resistance
Reactive oxygen species enhance mitochondrial function, insulin sensitivity and glucose uptake in skeletal muscle of senescence accelerated prone mice SAMP8.
Insulin Resistance
S6K1 regulates GSK3 under conditions of mTOR-dependent feedback inhibition of Akt.
Insulin Resistance
Short-term exercise training does not stimulate skeletal muscle ATP synthesis in relatives of humans with type 2 diabetes.
Insulin Resistance
Short-term high-fat diet induces muscle fiber type-selective anabolic resistance to resistance exercise.
Insulin Resistance
SIRT1 attenuates high glucose-induced insulin resistance via reducing mitochondrial dysfunction in skeletal muscle cells.
Insulin Resistance
SIRT1 overexpression in skeletal muscle in vivo induces increased insulin sensitivity and enhanced complex I but not complex II-V functions in individual subsarcolemmal and intermyofibrillar mitochondria.
Insulin Resistance
The Emerging Role of Branched-Chain Amino Acids in Insulin Resistance and Metabolism.
Insulin Resistance
The Relationship between Mitochondrial Dysfunction and Insulin Resistance in HIV-infected Children Receiving Antiretroviral Therapy.
Insulin Resistance
The signalling mechanisms of a novel mitochondrial complex I inhibitor prevent lipid accumulation and attenuate TNF-?-induced insulin resistance in vitro.
Insulin Resistance
Transcriptional profiles of type 2 diabetes in human skeletal muscle reveal insulin resistance, metabolic defects, apoptosis, and molecular signatures of immune activation in response to infections.
Insulinoma
Early loss of mammalian target of rapamycin complex 1 (mTORC1) signalling and reduction in cell size during dominant-negative suppression of hepatic nuclear factor 1-alpha (HNF1A) function in INS-1 insulinoma cells.
Insulinoma
Inhibition of mitochondrial complex I may account for IDDM induced by intoxication with the rodenticide Vacor.
Intellectual Disability
Bosch-Boonstra-Schaaf Optic Atrophy Syndrome Presenting as New-Onset Psychosis in a 32-Year-Old Man: A Case Report and Literature Review.
Intellectual Disability
Cerebellar ataxia, neuropathy, hearing loss, and intellectual disability due to AIFM1 mutation.
Intellectual Disability
Commentary on "Bosch-Boonstra-Schaaf Optic Atrophy Syndrome Presenting as New-Onset Psychosis in a 32-Year-Old Man: A Case Report and Literature Review".
Intellectual Disability
Heteroplasmic mitochondrial DNA 3310 mutation in NADH dehydrogenase subunit 1 associated with type 2 diabetes, hypertrophic cardiomyopathy, and mental retardation in a single patient.
Intellectual Disability
Mitochondrial myopathies with necrotizing encephalopathy of the Leigh type.
Intellectual Disability
Pharmacological intervention to restore connectivity deficits of neuronal networks derived from ASD patient iPSC with a TSC2 mutation.
Intellectual Disability
X-linked hypomyelination with spondylometaphyseal dysplasia (H-SMD) associated with mutations in AIFM1.
Iron Deficiencies
Dietary iron intake rapidly influences iron regulatory proteins, ferritin subunits and mitochondrial aconitase in rat liver.
Iron Deficiencies
Effect of Fe deficiency on mitochondrial alternative NAD(P)H dehydrogenases in cucumber roots.
Iron Deficiencies
Iron Deficiency without Anemia Decreases Physical Endurance and Mitochondrial Complex I Activity of Oxidative Skeletal Muscle in the Mouse.
Iron Deficiencies
Iron deficiency without anemia is responsible for decreased left ventricular function and reduced mitochondrial complex I activity in a mouse model.
Iron Deficiencies
mRNA-binding protein tristetraprolin is essential for cardiac response to iron deficiency by regulating mitochondrial function.
Iron Overload
Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease.
Iron Overload
Iron-induced energy supply deficiency and mitochondrial fragmentation in neurons.
Kearns-Sayre Syndrome
An autopsy case of mitochondrial encephalomyopathy with prominent degeneration in olivo-ponto-cerebellar system.
Kearns-Sayre Syndrome
Spontaneous Kearns-Sayre/chronic external ophthalmoplegia plus syndrome associated with a mitochondrial DNA deletion: a slip-replication model and metabolic therapy.
Keloid
Keloid Disease Can Be Inhibited by Antagonizing Excessive mTOR Signaling With a Novel Dual TORC1/2 Inhibitor.
Kidney Diseases
Rotenone remarkably attenuates oxidative stress, inflammation, and fibrosis in chronic obstructive uropathy.
Kidney Failure, Chronic
Podocytes maintain high basal levels of autophagy independent of mtor signaling.
Kidney Neoplasms
De novo design of thioredoxin reductase-targeted heterometallic titanocene-gold compounds of chlorambucil for mechanistic insights into renal cancer.
Kidney Neoplasms
Identification of alternatively spliced GRIM-19 mRNA in kidney cancer tissues.
Kidney Neoplasms
Integrated multi-omics characterization reveals a distinctive metabolic signature and the role of NDUFA4L2 in promoting angiogenesis, chemoresistance, and mitochondrial dysfunction in clear cell renal cell carcinoma.
Kidney Neoplasms
Phospholipase D-mTOR requirement for the Warburg effect in human cancer cells.
Kwashiorkor
Dietary Protein and Amino Acid Deficiency Inhibit Pancreatic Digestive Enzyme mRNA Translation by Multiple Mechanisms.
Laryngeal Neoplasms
Small interfering RNA survivin and GRIM-19 co-expression salmonella plasmid inhibited the growth of laryngeal cancer cells in vitro and in vivo.
Leigh Disease
A catalytic defect in mitochondrial respiratory chain complex I due to a mutation in NDUFS2 in a patient with Leigh syndrome.
Leigh Disease
A common pattern of brain MRI imaging in mitochondrial diseases with complex I deficiency.
Leigh Disease
A constant and similar assembly defect of mitochondrial respiratory chain complex I allows rapid identification of NDUFS4 mutations in patients with Leigh syndrome.
Leigh Disease
A novel mitochondrial m.14430A>G (MT-ND6, p.W82R) variant causes complex I deficiency and mitochondrial Leigh syndrome.
Leigh Disease
A novel mutation in the human complex I NDUFS7 subunit associated with Leigh syndrome.
Leigh Disease
A novel mutation of the NDUFS7 gene leads to activation of a cryptic exon and impaired assembly of mitochondrial complex I in a patient with Leigh syndrome.
Leigh Disease
A novel NDUFV1 gene mutation in complex I deficiency in consanguineous siblings with brainstem lesions and Leigh syndrome.
Leigh Disease
A novel recurrent mitochondrial DNA mutation in ND3 gene is associated with isolated complex I deficiency causing Leigh syndrome and dystonia.
Leigh Disease
A patient with homozygous nonsense variants in two Leigh syndrome disease genes: Distinguishing a dual diagnosis from a hypomorphic protein-truncating variant.
Leigh Disease
A Single Intravenous Injection of AAV-PHP.B-hNDUFS4 Ameliorates the Phenotype of Ndufs4
-/-
Mice.
Leigh Disease
Aberrant BCAA and glutamate metabolism linked to regional neurodegeneration in a mouse model of Leigh syndrome.
Leigh Disease
Application of the obligate aerobic yeast Yarrowia lipolytica as a eucaryotic model to analyse Leigh syndrome mutations in the complex I core subunits PSST and TYKY.
Leigh Disease
Association of the mtDNA m.4171C>A/MT-ND1 mutation with both optic neuropathy and bilateral brainstem lesions.
Leigh Disease
Bi-allelic pathogenic variants in NDUFC2 cause early-onset Leigh syndrome and stalled biogenesis of complex I.
Leigh Disease
Biallelic variants in two complex I genes cause abnormal splicing defects in probands with mild Leigh syndrome.
Leigh Disease
Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization.
Leigh Disease
Clinical presentation of mitochondrial respiratory chain defects in NADH-coenzyme Q reductase and cytochrome oxidase: clues to pathogenesis of Leigh disease.
Leigh Disease
Complex I (reduced nicotinamide-adenine dinucleotide-coenzyme Q reductase) deficiency in two patients with probable Leigh syndrome.
Leigh Disease
Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome.
Leigh Disease
Compound heterozygous missense and deep intronic variants in NDUFAF6 unraveled by exome sequencing and mRNA analysis.
Leigh Disease
De novo mutations in the mitochondrial ND3 gene as a cause of infantile mitochondrial encephalopathy and complex I deficiency.
Leigh Disease
Defective complex I assembly due to C20orf7 mutations as a new cause of Leigh syndrome.
Leigh Disease
Deletion of the Complex I Subunit NDUFS4 Adversely Modulates Cellular Differentiation.
Leigh Disease
Diagnosis of mitochondrial disorders by concomitant next-generation sequencing of the exome and mitochondrial genome.
Leigh Disease
Evolution of FOXRED1, an FAD-dependent oxidoreductase necessary for NADH:ubiquinone oxidoreductase (Complex I) assembly.
Leigh Disease
Evolution, structure and membrane association of NDUFAF6, an assembly factor for NADH:ubiquinone oxidoreductase (Complex I).
Leigh Disease
Exome sequencing identifies complex I NDUFV2 mutations as a novel cause of Leigh syndrome.
Leigh Disease
Fulminant neurological deterioration in a neonate with Leigh syndrome due to a maternally transmitted missense mutation in the mitochondrial ND3 gene.
Leigh Disease
Genotyping microsatellite DNA markers at putative disease loci in inbred/multiplex families with respiratory chain complex I deficiency allows rapid identification of a novel nonsense mutation (IVS1nt -1) in the NDUFS4 gene in Leigh syndrome.
Leigh Disease
Glutathione metabolism links FOXRED1 to NADH:ubiquinone oxidoreductase (complex I) deficiency: A hypothesis.
Leigh Disease
Hepatic S6K1 Partially Regulates Lifespan of Mice with Mitochondrial Complex I Deficiency.
Leigh Disease
Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome.
Leigh Disease
Identification of a deletion in the NDUFS4 gene using array-comparative genomic hybridization in a patient with suspected mitochondrial respiratory disease.
Leigh Disease
Impaired complex I assembly in a Leigh syndrome patient with a novel missense mutation in the ND6 gene.
Leigh Disease
Impaired hypoxic pulmonary vasoconstriction in a mouse model of Leigh syndrome.
Leigh Disease
Juvenile Leigh Syndrome, Optic Atrophy, Ataxia, Dystonia, and Epilepsy due to T14487C Mutation in the mtDNA-ND6 Gene: A Mitochondrial Syndrome Presenting From Birth to Adolescence.
Leigh Disease
Late-onset Leigh syndrome in a patient with mitochondrial complex I NDUFS8 mutations.
Leigh Disease
Leigh disease presenting in utero due to a novel missense mutation in the mitochondrial DNA-ND3.
Leigh Disease
Leigh disease with brainstem involvement in complex I deficiency due to assembly factor NDUFAF2 defect.
Leigh Disease
Leigh syndrome associated with a mutation in the NDUFS7 (PSST) nuclear encoded subunit of complex I.
Leigh Disease
Leigh syndrome associated with mitochondrial complex I deficiency due to a novel mutation in the NDUFS1 gene.
Leigh Disease
Leigh syndrome associated with mitochondrial complex I deficiency due to novel mutations In NDUFV1 and NDUFS2.
Leigh Disease
Leigh Syndrome Caused by the MT-ND5 m.13513G>A Mutation: A Case Presenting with WPW-Like Conduction Defect, Cardiomyopathy, Hypertension and Hyponatraemia.
Leigh Disease
Leigh Syndrome Mouse Model Can Be Rescued by Interventions that Normalize Brain Hyperoxia, but Not HIF Activation.
Leigh Disease
Leukodystrophy associated with mitochondrial complex I deficiency due to a novel mutation in the NDUFAF1 gene.
Leigh Disease
Long Survival in Patients With Leigh Syndrome and the m.10191T>C Mutation in MT-ND3: A Case Report and Review of the Literature.
Leigh Disease
Low mutant load of mitochondrial DNA G13513A mutation can cause Leigh's disease.
Leigh Disease
Metabolome and proteome profiling of complex I deficiency induced by rotenone.
Leigh Disease
Metformin delays neurological symptom onset in a mouse model of neuronal complex I deficiency.
Leigh Disease
Mitigation of NADH: ubiquinone oxidoreductase deficiency by chronic Trolox treatment.
Leigh Disease
Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase.
Leigh Disease
Mitochondrial complex I deficiency leads to the retardation of early embryonic development in Ndufs4 knockout mice.
Leigh Disease
Mitochondrial Complex I Mutations Predispose Drosophila to Isoflurane Neurotoxicity.
Leigh Disease
Mitochondrial DNA 11777C>A mutation associated Leigh syndrome: case report with a review of the previously described pedigrees.
Leigh Disease
Mitochondrial DNA mutations in Leigh syndrome and their phylogenetic implications.
Leigh Disease
Mitochondrial Proteome of Affected Glutamatergic Neurons in a Mouse Model of Leigh Syndrome.
Leigh Disease
Mitochondrial respiratory chain enzyme assay and DNA analysis in peripheral blood leukocytes for the etiological study of Chinese children with Leigh syndrome due to complex I deficiency.
Leigh Disease
Modulation of mitochondrial dysfunction-related oxidative stress in fibroblasts of patients with Leigh syndrome by inhibition of prooxidative p66Shc pathway.
Leigh Disease
MtDNA and nuclear mutations affecting oxidative phosphorylation: correlating severity of clinical defect with extent of bioenergetic compromise.
Leigh Disease
Mutated ND2 impairs mitochondrial complex I assembly and leads to Leigh syndrome.
Leigh Disease
Mutation in NDUFA13/GRIM19 leads to early onset hypotonia, dyskinesia and sensorial deficiencies, and mitochondrial complex I instability.
Leigh Disease
Mutation of mitochondrial DNA G13513A presenting with Leigh syndrome, Wolff-Parkinson-White syndrome and cardiomyopathy.
Leigh Disease
Mutations in mitochondrial complex I assembly factor NDUFAF3 cause Leigh syndrome.
Leigh Disease
Mutations in the gene encoding C8orf38 block complex I assembly by inhibiting production of the mitochondria-encoded subunit ND1.
Leigh Disease
Mutations in the NDUFS4 gene of mitochondrial complex I alter stability of the splice variants.
Leigh Disease
NAD+ Regeneration Rescues Lifespan, but Not Ataxia, in a Mouse Model of Brain Mitochondrial Complex I Dysfunction.
Leigh Disease
NDUFA10 mutations cause complex I deficiency in a patient with Leigh disease.
Leigh Disease
NDUFAF4 variants are associated with Leigh syndrome and cause a specific mitochondrial complex I assembly defect.
Leigh Disease
NDUFS4 deletion triggers loss of NDUFA12 in Ndufs4-/- mice and Leigh syndrome patients: A stabilizing role for NDUFAF2.
Leigh Disease
NDUFS4 mutations cause Leigh syndrome with predominant brainstem involvement.
Leigh Disease
NDUFS8-related Complex I Deficiency Extends Phenotype from "PEO Plus" to Leigh Syndrome.
Leigh Disease
Neonatal multiorgan failure due to ACAD9 mutation and complex I deficiency with mitochondrial hyperplasia in liver, cardiac myocytes, skeletal muscle, and renal tubules.
Leigh Disease
Novel Mitochondrial C15620A Variant may Modulate the Phenotype of Mitochondrial G11778A Mutation in a Chinese Family with Leigh Syndrome.
Leigh Disease
Novel NDUFA12 variants are associated with isolated complex I defect and variable clinical manifestation.
Leigh Disease
Ophthalmologic presentation of oxidative phosphorylation diseases of childhood.
Leigh Disease
Osteoblastic differentiation improved by bezafibrate-induced mitochondrial biogenesis in deciduous tooth-derived pulp stem cells from a child with Leigh syndrome.
Leigh Disease
Pathogenic Bi-allelic Mutations in NDUFAF8 Cause Leigh Syndrome with an Isolated Complex I Deficiency.
Leigh Disease
Phenotype-genotype correlations in Leigh syndrome: new insights from a multicentre study of 96 patients.
Leigh Disease
Photoreceptors in a mouse model of Leigh syndrome are capable of normal light-evoked signaling.
Leigh Disease
Progressive myoclonic epilepsy as an adult-onset manifestation of Leigh syndrome due to m.14487T>C.
Leigh Disease
Progressive myoclonic epilepsy due to rare mitochondrial ND6 mutation, m.14487T>C.
Leigh Disease
Quantifying small molecule phenotypic effects using mitochondrial morpho-functional fingerprinting and machine learning.
Leigh Disease
Rapid identification of a novel complex I MT-ND3 m.10134C>A mutation in a Leigh syndrome patient.
Leigh Disease
Region-Specific Defects of Respiratory Capacities in the Ndufs4(KO) Mouse Brain.
Leigh Disease
Respiratory chain complex I deficiency due to NDUFA12 mutations as a new cause of Leigh syndrome.
Leigh Disease
Sequence variations in the NDUFA1 gene encoding a subunit of complex I of the respiratory chain.
Leigh Disease
Severe infantile leigh syndrome associated with a rare mitochondrial ND6 mutation, m.14487T>C.
Leigh Disease
Succination is Increased on Select Proteins in the Brainstem of the NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) Knockout Mouse, a Model of Leigh Syndrome.
Leigh Disease
The 13042G --> A/ND5 mutation in mtDNA is pathogenic and can be associated also with a prevalent ocular phenotype.
Leigh Disease
The 8993 mtDNA mutation: heteroplasmy and clinical presentation in three families.
Leigh Disease
The clinical spectrum of the m.10191T>C mutation in complex I-deficient Leigh syndrome.
Leigh Disease
The first nuclear-encoded complex I mutation in a patient with Leigh syndrome.
Leigh Disease
The m.3890G>A/MT-ND1 mtDNA rare pathogenic variant: Expanding clinical and MRI phenotypes.
Leigh Disease
The mitochondria-targeted hydrogen sulfide donor AP39 improves health and mitochondrial function in a C. elegans primary mitochondrial disease model.
Leigh Disease
The mitochondrial 13513G > A mutation is most frequent in Leigh syndrome combined with reduced complex I activity, optic atrophy and/or Wolff-Parkinson-White.
Leigh Disease
The mitochondrial 13513G>A mutation is associated with Leigh disease phenotypes independent of complex I deficiency in muscle.
Leigh Disease
The mitochondrial DNA G13513A MELAS mutation in the NADH dehydrogenase 5 gene is a frequent cause of Leigh-like syndrome with isolated complex I deficiency.
Leigh Disease
The NDUFS4 Knockout Mouse: A Dual Threat Model of Childhood Mitochondrial Disease and Normative Aging.
Leigh Disease
The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families.
Leigh Disease
The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome.
Leigh Disease
Therapeutic applications of the TAT-mediated protein transduction system for complex I deficiency and other mitochondrial diseases.
Leigh Disease
Transition from Leigh syndrome to MELAS syndrome in a patient with heteroplasmic MT-ND3 m.10158T>C.
Leigh Disease
Two new mutations in the MT-TW gene leading to the disruption of the secondary structure of the tRNA(Trp) in patients with Leigh syndrome.
Leigh Disease
Widening the Heterogeneity of Leigh Syndrome: Clinical, Biochemical, and Neuroradiologic Features in a Patient Harboring a NDUFA10 Mutation.
Leigh Disease
[A case of Leigh syndrome associated with respiratory chain complex I deficiency due to mitochondrial gene 13513G>A mutation]
Leigh Disease
[Complex I and IV deficits in the mitochondrial respiratory chain in two siblings with type I glutaric aciduria]
Leigh Disease
[Leigh disease in an infant with deficiency of complex I of the mitochondrial respiratory chain]
Leiomyoma
Interactome Analysis Of The Differentially Expressed Proteins In Uterine Leiomyoma.
Leiomyosarcoma
Comparison of the therapeutic effects of two vanadium complexes administered at low dose on benzo[a]pyrene-induced malignant tumors in rats.
Leiomyosarcoma
Synthesis, structural characterization and biological studies of the triphenyltin(IV) complex with 2-thiobarbituric acid.
Leishmaniasis, Cutaneous
The population structure of Lutzomyia verrucarum (Diptera: Psycodidae), a Bartonella bacilliformis and Leishmania peruviana vector in Peru.
Lennox Gastaut Syndrome
Polyunsaturated fatty acid-enriched diet therapy for a child with epilepsy.
Leukemia
(+)?-Tocopheryl succinate inhibits the mitochondrial respiratory chain complex I and is as effective as arsenic trioxide or ATRA against acute promyelocytic leukemia in vivo.
Leukemia
A dinuclear monofunctional platinum(II) complex with an aromatic linker shows low reactivity towards glutathione but high DNA binding ability and antitumor activity.
Leukemia
A Nine-Coordinated Bismuth(III) Complex Derived from Pentadentate 2,6-Diacetylpyridine Bis((4)N-methylthiosemicarbazone): Crystal Structure and Both in Vitro and in Vivo Biological Evaluation.
Leukemia
A novel mechanism of action of methyl-2-cyano-3,12 dioxoolean-1,9 diene-28-oate: direct permeabilization of the inner mitochondrial membrane to inhibit electron transport and induce apoptosis.
Leukemia
Albumin prevents reactive oxygen species-induced mitochondrial damage, autophagy, and apoptosis during serum starvation.
Leukemia
B lymphoma Moloney murine leukemia virus insertion region 1: An oncogenic mediator in prostate cancer.
Leukemia
Biguanides sensitize leukemia cells to ABT-737-induced apoptosis by inhibiting mitochondrial electron transport.
Leukemia
BMI1 is downregulated by the natural compound curcumin, but not by bisdemethoxycurcumin and dimethoxycurcumin.
Leukemia
Data mining of NCI's anticancer screening database reveals mitochondrial complex I inhibitors cytotoxic to leukemia cell lines.
Leukemia
Degradation of the mitochondrial complex I assembly factor TMEM126B under chronic hypoxia.
Leukemia
Identification of BMI1 Promoter Inhibitors from Beaumontia murtonii and Eugenia operculata.
Leukemia
Inhibition of PCGF2 enhances granulocytic differentiation of acute promyelocytic leukemia cell line HL-60 via induction of HOXA7.
Leukemia
Knockdown BMI1 expression inhibits proliferation and invasion in human bladder cancer T24 cells.
Leukemia
NADH dehydrogenase deficiency in an apoptosis-resistant mutant isolated from a human HL-60 leukemia cell line.
Leukemia
Shikimic acid complexes of platinum. Preparation, reactivity, and antitumor activity of (R,R-1,2-diaminocyclohexane) bis(shikimato) platinum(II). Evidence for a novel rearrangement involving platinum-carbon bond formation.
Leukemia
Structure and biological properties of the copper(II) complex with the quinolone antibacterial drug N-propyl-norfloxacin and 2,2'-bipyridine.
Leukemia
Synthesis, crystal structures, in vitro biological evaluation of zinc(II) and bismuth(III) complexes of 2-acetylpyrazine N(4)-phenylthiosemicarbazone.
Leukemia
Tuning mTORC1 activity dictates the response of acute myeloid leukemia to LSD1 inhibition.
Leukemia, Erythroblastic, Acute
Apoptosis-inducing Factor, Mitochondrion-associated 2, Regulates Klf1 in a Mouse Erythroleukemia Cell Line.
Leukemia, Erythroblastic, Acute
Induction of Erythroid-Specific Expression in Murine Erythroleukemia (MEL) Cell Lines.
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Purification and characterization of a doxorubicin-inhibited NADH-quinone (NADH-ferricyanide) reductase from rat liver plasma membranes.
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Structure and biological properties of the copper(II) complex with the quinolone antibacterial drug N-propyl-norfloxacin and 2,2'-bipyridine.
Leukemia, Myeloid, Acute
CBF?-SMMHC Affects Genome-wide Polycomb Repressive Complex 1 Activity in Acute Myeloid Leukemia.
Leukemia, Myeloid, Acute
Cotargeting of Mitochondrial Complex I and Bcl-2 Shows Antileukemic Activity against Acute Myeloid Leukemia Cells Reliant on Oxidative Phosphorylation.
Leukemia, Myeloid, Acute
Inhibition of mammalian target of rapamycin signaling potentiates the effects of all-trans retinoic acid to induce growth arrest and differentiation of human acute myelogenous leukemia cells.
Leukemia, Myeloid, Acute
Mubritinib Targets the Electron Transport Chain Complex I and Reveals the Landscape of OXPHOS Dependency in Acute Myeloid Leukemia.
Leukemia, Myeloid, Acute
PI-103, a dual inhibitor of Class IA phosphatidylinositide 3-kinase and mTOR, has antileukemic activity in AML.
Leukemia, Myeloid, Acute
Prognostic implications and molecular associations of NADH dehydrogenase subunit 4 (ND4) mutations in acute myeloid leukemia.
Leukemia, Myeloid, Acute
Role of mTORC1-S6K1 signaling pathway in regulation of hematopoietic stem cell and acute myeloid leukemia.
Leukemia, Myeloid, Acute
Rotenone, a mitochondrial NADH dehydrogenase inhibitor, induces cell surface expression of CD13 and CD38 and apoptosis in HL-60 cells.
Leukemia, Myeloid, Acute
Sirolimus enhances remission induction in patients with high risk acute myeloid leukemia and mTORC1 target inhibition.
Leukemia, Myeloid, Acute
Targeting translation in acute myeloid leukemia: a new paradigm for therapy?
Leukemia, Myeloid, Acute
The Combined Treatment With the FLT3-Inhibitor AC220 and the Complex I Inhibitor IACS-010759 Synergistically Depletes Wt- and FLT3-Mutated Acute Myeloid Leukemia Cells.
Leukemia, Myeloid, Acute
The role of autophagy in targeted therapy for acute myeloid leukemia.
Leukemia, Promyelocytic, Acute
(+)?-Tocopheryl succinate inhibits the mitochondrial respiratory chain complex I and is as effective as arsenic trioxide or ATRA against acute promyelocytic leukemia in vivo.
Leukemia, Promyelocytic, Acute
Inhibition of PCGF2 enhances granulocytic differentiation of acute promyelocytic leukemia cell line HL-60 via induction of HOXA7.
Leukemia, Promyelocytic, Acute
The role of autophagy in targeted therapy for acute myeloid leukemia.
Leukemia, T-Cell
Deregulation of polycomb repressor complex 1 modifier AUTS2 in T-cell leukemia.
Leukodystrophy, Globoid Cell
The use of targeted genomic capture and massively parallel sequencing in diagnosis of Chinese Leukoencephalopathies.
Leukodystrophy, Metachromatic
The use of targeted genomic capture and massively parallel sequencing in diagnosis of Chinese Leukoencephalopathies.
Leukoencephalopathies
A novel mutation in the mitochondrial tRNA(Pro) gene associated with late-onset ataxia, retinitis pigmentosa, deafness, leukoencephalopathy and complex I deficiency.
Leukoencephalopathies
Clinical and molecular findings in children with complex I deficiency.
Leukoencephalopathies
Leukoencephalopathy with vanishing white matter caused by compound heterozygous mutations in mitochondrial complex I NDUFS1 subunit.
Leukoencephalopathies
MR spectroscopy and serial magnetic resonance imaging in a patient with mitochondrial cystic leukoencephalopathy due to complex I deficiency and NDUFV1 mutations and mild clinical course.
Leukoencephalopathies
NDUFAF3 variants that disrupt mitochondrial complex I assembly may associate with cavitating leukoencephalopathy.
Leukoencephalopathies
Pathogenic variants in NUBPL result in failure to assemble the matrix arm of complex I and cause a complex leukoencephalopathy with thalamic involvement.
Leukoencephalopathies
Progressive cavitating leukoencephalopathy associated with respiratory chain complex I deficiency and a novel mutation in NDUFS1.
Leukoencephalopathies
Recessive Mutations in NDUFA2 Cause Mitochondrial Leukoencephalopathy.
Leukoencephalopathies
Teaching NeuroImages: rapidly progressive leukoencephalopathy in mitochondrial complex I deficiency.
Leukoencephalopathies
The use of targeted genomic capture and massively parallel sequencing in diagnosis of Chinese Leukoencephalopathies.
Lewy Body Disease
Complex I reductions in the nucleus basalis of Meynert in Lewy body dementia: the role of Lewy bodies.
Lewy Body Disease
Oxidative stress as a cause of nigral cell death in Parkinson's disease and incidental Lewy body disease. The Royal Kings and Queens Parkinson's Disease Research Group.
Lipid Metabolism Disorders
Berberine alleviates lipid metabolism disorders via inhibition of mitochondrial complex I in gut and liver.
Lipoma
Identification of Novel Genes in Osteoarthritic Fibroblast-Like Synoviocytes Using Next-Generation Sequencing and Bioinformatics Approaches.
Liver Cirrhosis
TAK1-dependent autophagy: A suppressor of fatty liver disease and hepatic oncogenesis.
Liver Cirrhosis, Alcoholic
Ethanol sensitizes skeletal muscle to ammonia-induced molecular perturbations.
Liver Cirrhosis, Biliary
Mitochondrial autoantigens in primary biliary cirrhosis. Association of disease-specific determinants with a subunit of complex I (NADH-ubiquinone reductase) of the inner mitochondrial membrane.
Liver Diseases
A review of the studies on food-derived factors which regulate energy metabolism via the modulation of lipid-sensing nuclear receptors.
Liver Diseases
Highly expressed MT-ND3 positively associated with histological severity of hepatic steatosis.
Liver Diseases
Inhibition of tumor propellant glutathione peroxidase 4 induces ferroptosis in cancer cells and enhances anticancer effect of cisplatin.
Liver Diseases
Lipotoxicity-induced STING1 activation stimulates MTORC1 and restricts hepatic lipophagy.
Liver Diseases
Mitochondrial complex I subunits are decreased in murine nonalcoholic fatty liver disease: implication of peroxynitrite.
Liver Diseases
Obesity Challenges the Hepatoprotective Function of the Integrated Stress Response to Asparaginase Exposure in Mice.
Liver Diseases
SQSTM1/p62 activates NFE2L2/NRF2 via ULK1-mediated autophagic KEAP1 degradation and protects mouse liver from lipotoxicity.
Liver Diseases
TXNIP/VDUP1 attenuates steatohepatitis via autophagy and fatty acid oxidation.
Liver Failure
A high rate (20%-30%) of parental consanguinity in cytochrome-oxidase deficiency.
Liver Failure
Children's toxicology from bench to bed--Liver Injury (4): Mitochondrial respiratory chain disorder and liver disease in children.
Liver Failure
Infantile Progressive Hepatoencephalomyopathy with Combined OXPHOS Deficiency due to Mutations in the Mitochondrial Translation Elongation Factor Gene GFM1.
Liver Failure
Mitochondrial respiratory chain complex I deficiency with clinical and biochemical features of long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.
Liver Failure
Severe complex I deficiency in a case of neonatal-onset lactic acidosis and fatal liver failure.
Liver Neoplasms
A precision therapeutic strategy for hexokinase 1-null, hexokinase 2-positive cancers.
Liver Neoplasms
Activation of mTORC1 under nutrient starvation conditions increases cellular radiosensitivity in human liver cancer cell lines, HepG2 and HuH6.
Liver Neoplasms
Liver damage, inflammation, and enhanced tumorigenesis after persistent mTORC1 inhibition.
Liver Neoplasms
[Effect of invigorating spleen and detoxification decoction on MHC I/MHC II in spleen-deficiency liver cancer rats survival].
Lung Diseases
Estrogen activates pyruvate kinase M2 and increases the growth of TSC2-deficient cells.
Lung Diseases
Integration of mTOR and estrogen-ERK2 signaling in lymphangioleiomyomatosis pathogenesis.
Lung Diseases
Metabolic Changes in Patients With Lymphangioleiomyomatosis Treated With Sirolimus and Hydroxychloroquine.
Lung Diseases
mTORC1 activation in lung mesenchyme drives sex- and age-dependent pulmonary structure and function decline.
Lung Diseases
Rationally Designed 3D Hydrogels Model Invasive Lung Diseases Enabling High-Content Drug Screening.
Lung Diseases, Interstitial
Inhibition of mTORC1 induces loss of E-cadherin through AKT/GSK-3? signaling-mediated upregulation of E-cadherin repressor complexes in non-small cell lung cancer cells.
Lung Injury
LFA-1 and MAC-1 mediate pulmonary recruitment of neutrophils and tissue damage in abdominal sepsis.
Lung Injury
Mitochondrial peptides cause proinflammatory responses in the alveolar epithelium via FPR-1, MAPKs, and AKT: a potential mechanism involved in acute lung injury.
Lung Injury
Mitochondrial respiratory complex I regulates neutrophil activation and severity of lung injury.
Lung Neoplasms
A dinuclear monofunctional platinum(II) complex with an aromatic linker shows low reactivity towards glutathione but high DNA binding ability and antitumor activity.
Lung Neoplasms
Activation of AIFM2 enhances apoptosis of human lung cancer cells undergoing toxicological stress.
Lung Neoplasms
Association of predicted pathogenic mutations in mitochondrial ND genes with distant metastasis in NSCLC and colon cancer.
Lung Neoplasms
Axial functionalisation of photoactive diazido platinum(iv) anticancer complexes.
Lung Neoplasms
Biomolecular Interaction, Anti-Cancer and Anti-Angiogenic Properties of Cobalt(III) Schiff Base Complexes.
Lung Neoplasms
Combination treatment with perifosine and MEK-162 demonstrates synergism against lung cancer cells in vitro and in vivo.
Lung Neoplasms
Half-Sandwich Iridium and Ruthenium Complexes: Effective Tracking in Cells and Anticancer Studies.
Lung Neoplasms
Highly Promising Antitumor Agent of a Novel Platinum(II) Complex Bearing a Tetradentate Chelating Ligand.
Lung Neoplasms
HORMAD1 is a negative prognostic indicator in lung adenocarcinoma and specifies resistance to oxidative and genotoxic stress.
Lung Neoplasms
IMPAD1 functions as mitochondrial electron transport inhibitor that prevents ROS production and promotes lung cancer metastasis through the AMPK-Notch1-HEY1 pathway.
Lung Neoplasms
MCT4 is induced by metastasis-enhancing pathogenic mitochondrial NADH dehydrogenase gene mutations and can be a therapeutic target.
Lung Neoplasms
Mitochondria-Targeted Honokiol Confers a Striking Inhibitory Effect on Lung Cancer via Inhibiting Complex I Activity.
Lung Neoplasms
Oxoplatin-B, a cisplatin-based platinum(IV) complex with photoactive BODIPY for mitochondria specific "chemo-PDT" activity.
Lung Neoplasms
PAQR3 suppresses the growth of non-small cell lung cancer cells via modulation of EGFR-mediated autophagy.
Lung Neoplasms
Programmed death ligand 1-associated antitumor immunity in early-stage non-small cell lung cancer: Expression of major histocompatibility complex I as a prerequisite.
Lung Neoplasms
S100A4 alters metabolism and promotes invasion of lung cancer cells by up-regulating mitochondrial complex I protein NDUFS2.
Lung Neoplasms
Synthesis, crystal structures, DNA binding and cytotoxicity of two novel platinum(II) complexes containing 2-(hydroxymethyl)pyridine and pyridine-2-carboxylate ligands.
Lung Neoplasms
Target Discovery of Selective Non-Small-Cell Lung Cancer Toxins Reveals Inhibitors of Mitochondrial Complex I.
Lung Neoplasms
Targeting Human Lung Adenocarcinoma with a Suppressor of Mitochondrial Superoxide Production.
Lung Neoplasms
The anti-cancer drug ABTL0812 induces ER stress-mediated cytotoxic autophagy by increasing dihydroceramide levels in cancer cells.
Lung Neoplasms
The Effect on Cognition of Mitochondrial Respiratory System Proteins in Peripheral Blood Mononuclear Cells in the Course of Lung Cancer.
Lung Neoplasms
The opposite prognostic effect of NDUFS1 and NDUFS8 in lung cancer reflects the oncojanus role of mitochondrial complex I.
Lung Neoplasms
The role of bridging ligands in determining DNA-binding ability and cross-linking patterns of dinuclear platinum(II) antitumour complexes.
Lung Neoplasms
[Expression and clinical significance of GRIM-19 in non-small cell lung cancer.]
Lupus Erythematosus, Systemic
INCREASED MITOCHONDRIAL ELECTRON TRANSPORT CHAIN ACTIVITY AT COMPLEX I IS REGULATED BY N-ACETYLCYSTEINE IN LYMPHOCYTES OF PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS.
Lupus Erythematosus, Systemic
Mechanistic Target of Rapamycin Complex 1 Expands Th17 and IL-4+ CD4-CD8- Double-Negative T Cells and Contracts Regulatory T Cells in Systemic Lupus Erythematosus.
Lymphangioleiomyomatosis
Connectivity Map Analysis of a Single-Cell RNA-Sequencing -Derived Transcriptional Signature of mTOR Signaling.
Lymphangioleiomyomatosis
Estrogen activates pyruvate kinase M2 and increases the growth of TSC2-deficient cells.
Lymphangioleiomyomatosis
Integration of mTOR and estrogen-ERK2 signaling in lymphangioleiomyomatosis pathogenesis.
Lymphangioleiomyomatosis
Interleukin-6 mediates PSAT1 expression and serine metabolism in TSC2-deficient cells.
Lymphangioleiomyomatosis
Lymphangioleiomyomatosis Biomarkers Linked to Lung Metastatic Potential and Cell Stemness.
Lymphangioleiomyomatosis
Metabolic Changes in Patients With Lymphangioleiomyomatosis Treated With Sirolimus and Hydroxychloroquine.
Lymphangioleiomyomatosis
mTORC1 activation in lung mesenchyme drives sex- and age-dependent pulmonary structure and function decline.
Lymphangioleiomyomatosis
Pharmacological inhibition of Polo-like kinase 1 (PLK1) by BI-2536 decreases the viability and survival of hamartin and tuberin deficient cells via induction of apoptosis and attenuation of autophagy.
Lymphangioleiomyomatosis
Resveratrol prevents rapamycin-induced upregulation of autophagy and selectively induces apoptosis in TSC2-deficient cells.
Lymphangioleiomyomatosis
YAP enters the mTOR pathway to promote tuberous sclerosis complex.
Lymphatic Metastasis
Down-regulation of GRIM-19 is associated with STAT3 overexpression in breast carcinomas.
Lymphatic Metastasis
GRIM-19 Restores Cervical Cancer Cell Senescence by Repressing hTERT Transcription.
Lymphatic Metastasis
Mitochondrial GRIM-19 as a potential therapeutic target for STAT3-dependent carcinogenesis of gastric cancer.
Lymphatic Metastasis
Nonsense and missense mutation of mitochondrial ND6 gene promotes cell migration and invasion in human lung adenocarcinoma.
Lymphocytic Choriomeningitis
Antigen presentation and cytotoxic T lymphocyte killing studied in individual, living cells.
Lymphocytic Choriomeningitis
Mammalian target of rapamycin complex 1 signalling is essential for germinal centre reaction.
Lymphocytic Choriomeningitis
RNAi screening reveals requirement for host cell secretory pathway in infection by diverse families of negative-strand RNA viruses.
Lymphoma
Apurinic/apyrimidinic endonuclease/redox factor 1 (APE1) alleviates myocardial hypoxia-reoxygenation injury by inhibiting oxidative stress and ameliorating mitochondrial dysfunction.
Lymphoma
B lymphoma Moloney murine leukemia virus insertion region 1: An oncogenic mediator in prostate cancer.
Lymphoma
BMI1 Deficiency Results in Female Infertility by Activating p16/p19 Signaling and Increasing Oxidative Stress.
Lymphoma
Comparative analysis of testis transcriptomes associated with male infertility in triploid cyprinid fish.
Lymphoma
Control of B-cell lymphoma by therapeutic vaccination and acquisition of immune resistance is independent of direct tumour IFN-gamma signalling.
Lymphoma
Discovery of a strongly apoptotic ruthenium complex through combinatorial coordination chemistry.
Lymphoma
Dual mTORC1/mTORC2 inhibition diminishes Akt activation and induces Puma-dependent apoptosis in lymphoid malignancies.
Lymphoma
Essential role of glucokinase in the protection of pancreatic ? cells to the glucose energetic status.
Lymphoma
Inhibition of monocarboxyate transporter 1 by AZD3965 as a novel therapeutic approach for diffuse large B-cell lymphoma and Burkitt lymphoma.
Lymphoma
Mitochondrial complex II prevents hypoxic but not calcium- and proapoptotic Bcl-2 protein-induced mitochondrial membrane potential loss.
Lymphoma
mTORC1 inhibitors: is temsirolimus in renal cancer telling us how they really work?
Lymphoma
Regulation of gene transcription of B lymphoma Mo-MLV insertion region 1 homolog (Review).
Lymphoma, B-Cell
Apurinic/apyrimidinic endonuclease/redox factor 1 (APE1) alleviates myocardial hypoxia-reoxygenation injury by inhibiting oxidative stress and ameliorating mitochondrial dysfunction.
Lymphoma, B-Cell
Essential role of glucokinase in the protection of pancreatic ? cells to the glucose energetic status.
Lymphoma, B-Cell
Everolimus combined with R-CHOP-21 for new, untreated, diffuse large B-cell lymphoma (NCCTG 1085 [Alliance]): safety and efficacy results of a phase 1 and feasibility trial.
Lymphoma, B-Cell
Evidence of altered depression and dementia-related proteins in the brains of young rats after ovariectomy.
Lymphoma, B-Cell
Identification of collaborative activities with oxidative phosphorylation in bipolar disorder.
Lymphoma, B-Cell
Inhibition of monocarboxyate transporter 1 by AZD3965 as a novel therapeutic approach for diffuse large B-cell lymphoma and Burkitt lymphoma.
Lymphoma, Large B-Cell, Diffuse
Everolimus combined with R-CHOP-21 for new, untreated, diffuse large B-cell lymphoma (NCCTG 1085 [Alliance]): safety and efficacy results of a phase 1 and feasibility trial.
Lymphoma, Large B-Cell, Diffuse
Inhibition of monocarboxyate transporter 1 by AZD3965 as a novel therapeutic approach for diffuse large B-cell lymphoma and Burkitt lymphoma.
Lymphoma, Large B-Cell, Diffuse
mTOR activity in AIDS-related diffuse large B-cell lymphoma.
Lymphoma, Mantle-Cell
Dual mTORC1/mTORC2 inhibition diminishes Akt activation and induces Puma-dependent apoptosis in lymphoid malignancies.
Lymphoma, Non-Hodgkin
Combinations of mTORC1 inhibitor RAD001 with gemcitabine and paclitaxel for treating non-Hodgkin lymphoma.
Lymphoma, T-Cell, Cutaneous
Retraction: Simultaneous Inhibition of mTOR-Containing Complex 1 (mTORC1) and MNK Induces Apoptosis of Cutaneous T-Cell Lymphoma (CTCL) Cells.
Lymphoma, T-Cell, Cutaneous
Simultaneous inhibition of mTOR-containing complex 1 (mTORC1) and MNK induces apoptosis of cutaneous T-cell lymphoma (CTCL) cells.
Lysosomal Storage Diseases
Contractile activity attenuates autophagy suppression and reverses mitochondrial defects in skeletal muscle cells.
Machado-Joseph Disease
Mitochondrial NADH Dehydrogenase Subunit 3 Polymorphism Associated with an Earlier Age at Onset in Male Machado-Joseph disease Patients.
Macular Degeneration
Mitochondrial DNA variants of respiratory complex I that uniquely characterize haplogroup T2 are associated with increased risk of age-related macular degeneration.
Malaria
An anchored restriction-mapping approach applied to the genetic analysis of the Anopheles gambiae malaria vector complex 1.
Malaria
Functional characterization and target validation of alternative complex I of Plasmodium falciparum mitochondria.
Malaria
Identification, Design and Biological Evaluation of Bisaryl Quinolones Targeting Plasmodium falciparum Type II NADH:Quinone Oxidoreductase (PfNDH2).
Malaria
Identification, Design and Biological Evaluation of Heterocyclic Quinolones Targeting Plasmodium falciparum Type II NADH:Quinone Oxidoreductase (PfNDH2).
Malaria
Mitochondrial type II NADH dehydrogenase of Plasmodium falciparum (PfNDH2) is dispensable in the asexual blood stages.
Malaria
New insecticide screening platforms indicate that Mitochondrial Complex I inhibitors are susceptible to cross-resistance by mosquito P450s that metabolise pyrethroids.
Malaria
Target Elucidation by Cocrystal Structures of NADH-Ubiquinone Oxidoreductase of Plasmodium falciparum (PfNDH2) with Small Molecule To Eliminate Drug-Resistant Malaria.
Malaria
The malaria parasite type II NADH:quinone oxidoreductase: an alternative enzyme for an alternative lifestyle.
Malaria
The structure of the yeast NADH dehydrogenase (Ndi1) reveals overlapping binding sites for water- and lipid-soluble substrates.
Malformations of Cortical Development
A human minisatellite hosts an alternative transcription start site for NPRL3 driving its expression in a repeat number-dependent manner.
Malformations of Cortical Development
Contribution of Altered Endocannabinoid System to Overactive mTORC1 Signaling in Focal Cortical Dysplasia.
Malformations of Cortical Development
Inhibition of the mammalian target of rapamycin blocks epilepsy progression in NS-Pten conditional knockout mice.
Malformations of Cortical Development
No evidence for human papillomavirus infection in focal cortical dysplasia IIb.
Malnutrition
A low pretransplant peripheral blood mononuclear cell complex I activity predicts metabolic disturbances and inability to regain fat free mass in cirrhotic patients undergoing liver transplantation.
Malnutrition
Effect of feeding malnourished patients for 1 mo on mitochondrial complex I activity and nutritional assessment measurements.
Malnutrition
Effect of malnutrition and short-term refeeding on peripheral blood mononuclear cell mitochondrial complex I activity in humans.
Malnutrition
Impaired interferon signaling in chronic hepatitis C patients with advanced fibrosis via the transforming growth factor beta signaling pathway.
Malnutrition
Relationship between metabolism and peripheral blood mononuclear cell mitochondrial complex I activity before and after a short-term refeeding in weight-losing cancer patients.
Malocclusion
MTORC1 coordinates the autophagy and apoptosis signaling in articular chondrocytes in osteoarthritic temporomandibular joint.
Mania
Mitochondrial dysfunctions in blood platelets of patients with manic episode of bipolar disorder`.
Massive Hepatic Necrosis
Rotenone, a mitochondrial respiratory complex I inhibitor, ameliorates lipopolysaccharide/D-galactosamine-induced fulminant hepatitis in mice.
Megalencephaly
Contribution of Altered Endocannabinoid System to Overactive mTORC1 Signaling in Focal Cortical Dysplasia.
Megalencephaly
Necrotizing encephalopathy and macrocephaly with mitochondrial complex I deficiency.
Megalencephaly
New familial mitochondrial encephalopathy with macrocephaly, cardiomyopathy, and complex I deficiency.
Melanoma
3-Methyladenine prevents energy stress-induced necrotic death of melanoma cells through autophagy-independent mechanisms.
Melanoma
A novel mitochondrial inhibitor blocks MAPK pathway and overcomes MAPK inhibitor-resistance in melanoma.
Melanoma
Activation of AMP-activated protein kinase is involved in vincristine-induced cell apoptosis in B16 melanoma cell.
Melanoma
Bladder cancer cell-intrinsic PD-L1 signals promote mTOR and autophagy activation that can be inhibited to improve cytotoxic chemotherapy.
Melanoma
CD147 interacts with NDUFS6 in regulating mitochondrial complex I activity and the mitochondrial apoptotic pathway in human malignant melanoma cells.
Melanoma
Compound 13, an ?1-selective small molecule activator of AMPK, potently inhibits melanoma cell proliferation.
Melanoma
Dual Targeting of BRAF and mTOR Signaling in Melanoma Cells with Pyridinyl Imidazole Compounds.
Melanoma
Identification of an iridium(III) complex with anti-bacterial and anti-cancer activity.
Melanoma
Inhibition of mitochondrial respiration prevents BRAF-mutant melanoma brain metastasis.
Melanoma
Loss of polycomb repressive complex 1 activity and chromosomal instability drive uveal melanoma progression.
Melanoma
Mitochondrial complex I inhibition triggers a mitophagy-dependent ROS increase leading to necroptosis and ferroptosis in melanoma cells.
Melanoma
Mitochondrial complex I inhibitor deguelin induces metabolic reprogramming and sensitizes vemurafenib-resistant BRAFV600E mutation bearing metastatic melanoma cells.
Melanoma
Mitochondrial Oxidative Stress due to Complex I Dysfunction Promotes Fibroblast Activation and Melanoma Cell Invasiveness.
Melanoma
Positive Feedback Loop Between PI3K-Akt-mTORC1 Signaling and the Lipogenic Pathway Boosts Akt Signaling: Induction of the Lipogenic Pathway by a Melanoma Antigen.
Melanoma
Simultaneous blocking of the pan-RAF and S100B pathways as a synergistic therapeutic strategy against malignant melanoma.
Melanoma
Synchronous effects of targeted mitochondrial complex I inhibitors on tumor and immune cells abrogate melanoma progression.
Melanoma
Synthesis, Spectral Characterization, DNA/ Protein Binding, DNA Cleavage, Cytotoxicity, Antioxidative and Molecular Docking Studies of Cu(II)Complexes Containing Schiff Base-bpy/Phen Ligands.
Melanoma
Targeting metabolic flexibility by simultaneously inhibiting respiratory complex I and lactate generation retards melanoma progression.
Melanoma
Targeting mitochondrial complex I using BAY 87-2243 reduces melanoma tumor growth.
Melanoma
The role of ancillary ligand substituents in the biological activity of triruthenium-NO complexes.
Melanoma
The water-soluble Roussin's red ester acting as a potential photochemical NO-delivery agent: photolysis reactions, DNA cleavage and anticancer activity.
Melanoma
Unlike reactivity of mono- and binuclear imine-copper(II) complexes toward melanoma cells via a tyrosinase-dependent mechanism.
Melanoma
ZNNT1 long noncoding RNA induces autophagy to inhibit tumorigenesis of uveal melanoma by regulating key autophagy gene expression.
Melanoma, Experimental
Activation of AMP-activated protein kinase is involved in vincristine-induced cell apoptosis in B16 melanoma cell.
Melanoma, Experimental
Enhancing Radiation Therapy Through Cherenkov Light-Activated Phototherapy.
MELAS Syndrome
A False-Positive Diagnosis for the Common MELAS (A3243G) Mutation Caused by a Novel Variant (A3426G) in the ND1 Gene of Mitochondria DNA.
MELAS Syndrome
A novel mitochondrial ND5 (MTND5) gene mutation giving isolated exercise intolerance.
MELAS Syndrome
A novel mtDNA mutation in the ND5 subunit of complex I in two MELAS patients.
MELAS Syndrome
Accumulation of mtDNA with a mutation at position 3271 in tRNA(Leu)(UUR) gene introduced from a MELAS patient to HeLa cells lacking mtDNA results in progressive inhibition of mitochondrial respiratory function.
MELAS Syndrome
An mtDNA mutation, 14453G-->A, in the NADH dehydrogenase subunit 6 associated with severe MELAS syndrome.
MELAS Syndrome
Codon-specific translational defect caused by a wobble modification deficiency in mutant tRNA from a human mitochondrial disease.
MELAS Syndrome
Complex I (reduced nicotinamide-adenine dinucleotide-coenzyme Q reductase) deficiency in two patients with probable Leigh syndrome.
MELAS Syndrome
Complex I deficiency is associated with 3243G:C mitochondrial DNA in osteosarcoma cell cybrids.
MELAS Syndrome
Immunohistochemical analysis of the oxidative phosphorylation complexes in skeletal muscle from patients with mitochondrial DNA encoded tRNA gene defects.
MELAS Syndrome
Is the mitochondrial complex I ND5 gene a hot-spot for MELAS causing mutations?
MELAS Syndrome
Leber hereditary optic neuropathy and dystonia overlapping mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes due to m.14459G>A mutation.
MELAS Syndrome
LHON/MELAS overlap mutation in ND1 subunit of mitochondrial complex I affects ubiquinone binding as revealed by modeling in Escherichia coli NDH-1.
MELAS Syndrome
LHON/MELAS overlap syndrome associated with a mitochondrial MTND1 gene mutation.
MELAS Syndrome
Mitochondrial DNA polymorphism in disease: a possible contributor to respiratory dysfunction.
MELAS Syndrome
Mitochondrial encephalomyopathies: a correlation between neuropathological findings and defects in mitochondrial DNA.
MELAS Syndrome
Mitochondrial ND5 gene variation associated with encephalomyopathy and mitochondrial ATP consumption.
MELAS Syndrome
Mitochondrial variations in the MT-ND4 and MT-TL1 genes are associated with male infertility.
MELAS Syndrome
Progression from MERRF to MELAS phenotype in a patient with combined respiratory complex I and IV deficiencies.
MELAS Syndrome
The 13042G --> A/ND5 mutation in mtDNA is pathogenic and can be associated also with a prevalent ocular phenotype.
MELAS Syndrome
The accumulation of assembly intermediates of the mitochondrial complex I matrix arm is reduced by limiting glucose uptake in a neuronal-like model of MELAS syndrome.
MELAS Syndrome
The addition of ketone bodies alleviates mitochondrial dysfunction by restoring complex I assembly in a MELAS cellular model.
MELAS Syndrome
The G13513A mutation in the ND5 gene of mitochondrial DNA as a common cause of MELAS or Leigh syndrome: evidence from 12 cases.
MELAS Syndrome
The m.3243A>G mtDNA mutation is pathogenic in an in vitro model of the human blood brain barrier.
MELAS Syndrome
The MELAS mutations 3946 and 3949 perturb the critical structure in a conserved loop of the ND1 subunit of mitochondrial complex I.
MELAS Syndrome
The mitochondrial 13513G>A mutation is associated with Leigh disease phenotypes independent of complex I deficiency in muscle.
MELAS Syndrome
The mitochondrial DNA G13513A MELAS mutation in the NADH dehydrogenase 5 gene is a frequent cause of Leigh-like syndrome with isolated complex I deficiency.
MELAS Syndrome
The role of complex I genes in MELAS: a novel heteroplasmic mutation 3380G>A in ND1 of mtDNA.
MELAS Syndrome
Tissue- and cell-type-specific manifestations of heteroplasmic mtDNA 3243A>G mutation in human induced pluripotent stem cell-derived disease model.
MELAS Syndrome
Transition from Leigh syndrome to MELAS syndrome in a patient with heteroplasmic MT-ND3 m.10158T>C.
MELAS Syndrome
Two cases of NADH-coenzyme Q reductase deficiency: relationship to MELAS syndrome.
MELAS Syndrome
Two mtDNA mutations 14487T>C (M63V, ND6) and 12297T>C (tRNA Leu) in a Leigh syndrome family.
MELAS Syndrome
[Complex I (NADH coenzyme-Q-reductase) deficiency, MELAS syndrome and hypertrophic cardiomyopathy]
Memory Disorders
Regulation of filial imprinting and structural plasticity by mTORC1 in newborn chickens.
Meningioma
Alterations of oxidative phosphorylation in meningiomas and peripheral nerve sheath tumors.
Meningioma
mTOR kinase inhibition disrupts neuregulin 1-ERBB3 autocrine signaling and sensitizes NF2-deficient meningioma cellular models to IGF1R inhibition.
Meningioma
NF2/merlin is a novel negative regulator of mTOR complex 1, and activation of mTORC1 is associated with meningioma and schwannoma growth.
Meningioma
Regulation of mTOR complex 2 signaling in neurofibromatosis 2-deficient target cell types.
Meningitis
Evaluation of mitochondrial respiratory chain in the brain of rats after pneumococcal meningitis.
Menkes Kinky Hair Syndrome
Atypical form of Menkes kinky hair disease with mitochondrial NADH-CoQ reductase deficiency.
MERRF Syndrome
Immunohistochemical analysis of the oxidative phosphorylation complexes in skeletal muscle from patients with mitochondrial DNA encoded tRNA gene defects.
MERRF Syndrome
Mitochondrial DNA polymorphism in disease: a possible contributor to respiratory dysfunction.
MERRF Syndrome
Myoclonic epilepsy with ragged-red fibers (MERRF): an immunohistochemical study of the brain.
MERRF Syndrome
Progression from MERRF to MELAS phenotype in a patient with combined respiratory complex I and IV deficiencies.
Mesothelioma
Spreading of mesothelioma cells is rapamycin-sensitive and requires continuing translation.
Metabolic Diseases
Loss of mTORC1 signalling impairs ?-cell homeostasis and insulin processing.
Metabolic Diseases
N-Formyl-3,4-methylenedioxy-benzylidene-gamma-butyrolaetam, KNK437 induces caspase-3 activation through inhibition of mTORC1 activity in Cos-1 cells.
Metabolic Diseases
Quercetin preserves redox status and stimulates mitochondrial function in metabolically-stressed HepG2 cells.
Metabolic Diseases
Redefining the roles of mitochondrial DNA-encoded subunits in respiratory Complex I assembly.
Metabolic Syndrome
A mechanism underlying hypertensive occurrence in the metabolic syndrome: cooperative effect of oxidative stress and calcium accumulation in vascular smooth muscle cells.
Metabolic Syndrome
Metabolic effects of RUBCN/Rubicon deficiency in kidney proximal tubular epithelial cells.
Methemoglobinemia
Heterologous expression of enzymopenic methemoglobinemia variants using a novel NADH:cytochrome c reductase fusion protein.
Microcephaly
A homozygous variant in NDUFA8 is associated with developmental delay, microcephaly, and epilepsy due to mitochondrial complex I deficiency.
Microphthalmos
Histiocytoid cardiomyopathy and microphthalmia with linear skin defects syndrome: phenotypes linked by truncating variants in NDUFB11.
Microphthalmos
Lysosome Alterations in the Human Epithelial Cell Line HaCaT and Skin Specimens: Relevance to Psoriasis.
Microphthalmos
Mutations in NDUFB11, encoding a complex I component of the mitochondrial respiratory chain, cause microphthalmia with linear skin defects syndrome.
Microphthalmos
WIPI1 Coordinates Melanogenic Gene Transcription and Melanosome Formation via TORC1 Inhibition.
Migraine Disorders
Progressive myoclonic epilepsy due to rare mitochondrial ND6 mutation, m.14487T>C.
Migraine with Aura
Progressive myoclonic epilepsy due to rare mitochondrial ND6 mutation, m.14487T>C.
Mitochondrial Diseases
5q14.3 Deletion Manifesting as Mitochondrial Disease and Autism: Case Report.
Mitochondrial Diseases
A common pattern of brain MRI imaging in mitochondrial diseases with complex I deficiency.
Mitochondrial Diseases
A Diagnostic Algorithm for Mitochondrial Disorders in Estonian Children.
Mitochondrial Diseases
A disease-associated Aifm1 variant induces severe myopathy in knockin mice.
Mitochondrial Diseases
A Drosophila model of mitochondrial disease caused by a complex I mutation that uncouples proton pumping from electron transfer.
Mitochondrial Diseases
A homozygous variant in NDUFA8 is associated with developmental delay, microcephaly, and epilepsy due to mitochondrial complex I deficiency.
Mitochondrial Diseases
A novel AIFM1 mutation expands the phenotype to an infantile motor neuron disease.
Mitochondrial Diseases
A novel mitochondrial MTND5 frameshift mutation causing isolated complex I deficiency, renal failure and myopathy.
Mitochondrial Diseases
A novel mutation in the human complex I NDUFS7 subunit associated with Leigh syndrome.
Mitochondrial Diseases
A novel mutation in the thiamine responsive megaloblastic anaemia gene SLC19A2 in a patient with deficiency of respiratory chain complex I.
Mitochondrial Diseases
A novel mutation of the NDUFS7 gene leads to activation of a cryptic exon and impaired assembly of mitochondrial complex I in a patient with Leigh syndrome.
Mitochondrial Diseases
A novel recurrent mitochondrial DNA mutation in ND3 gene is associated with isolated complex I deficiency causing Leigh syndrome and dystonia.
Mitochondrial Diseases
A recurrent mitochondrial p.Trp22Arg NDUFB3 variant causes a distinctive facial appearance, short stature and a mild biochemical and clinical phenotype.
Mitochondrial Diseases
A simplified and reliable assay for complex I in human blood lymphocytes.
Mitochondrial Diseases
Aberrant BCAA and glutamate metabolism linked to regional neurodegeneration in a mouse model of Leigh syndrome.
Mitochondrial Diseases
Acadian variant of Fanconi syndrome is caused by mitochondrial respiratory chain complex I deficiency due to a non-coding mutation in complex I assembly factor NDUFAF6.
Mitochondrial Diseases
Accessory subunits are integral for assembly and function of human mitochondrial complex I.
Mitochondrial Diseases
AIFM1 mutation presenting with fatal encephalomyopathy and mitochondrial disease in an infant.
Mitochondrial Diseases
An X-chromosome linked mouse model (Ndufa1(S55A)) for systemic partial Complex I deficiency for studying predisposition to neurodegeneration and other diseases.
Mitochondrial Diseases
Anaesthetic considerations for a 2-month-old infant with suspected complex I respiratory chain deficiency.
Mitochondrial Diseases
Analysis of Human Mutations in the Supernumerary Subunits of Complex I.
Mitochondrial Diseases
Analysis of the mitochondrial encoded subunits of complex I in 20 patients with a complex I deficiency.
Mitochondrial Diseases
Anesthesia for a child with complex I respiratory chain enzyme deficiency.
Mitochondrial Diseases
Anesthetic Hypersensitivity in a Case-Controlled Series of Patients With Mitochondrial Disease.
Mitochondrial Diseases
Antioxidants partially restore glutamate transport defect in leber hereditary optic neuropathy cybrids.
Mitochondrial Diseases
Apoptosis inducing factor deficiency causes retinal photoreceptor degeneration. The protective role of the redox compound methylene blue.
Mitochondrial Diseases
Arabidopsis thaliana alternative dehydrogenases: a potential therapy for mitochondrial complex I deficiency? Perspectives and pitfalls.
Mitochondrial Diseases
Assay of mitochondrial respiratory chain complex I in human lymphocytes and cultured skin fibroblasts.
Mitochondrial Diseases
Assembly defects induce oxidative stress in inherited mitochondrial complex I deficiency.
Mitochondrial Diseases
Bi-allelic Mutations in NDUFA6 Establish Its Role in Early-Onset Isolated Mitochondrial Complex I Deficiency.
Mitochondrial Diseases
Bi-allelic pathogenic variants in NDUFC2 cause early-onset Leigh syndrome and stalled biogenesis of complex I.
Mitochondrial Diseases
Biallelic Mutations in TMEM126B Cause Severe Complex I Deficiency with a Variable Clinical Phenotype.
Mitochondrial Diseases
Bioactivity and gene expression profiles of hiPSC-generated retinal ganglion cells in MT-ND4 mutated Leber's hereditary optic neuropathy.
Mitochondrial Diseases
Bipolar cell reduction precedes retinal ganglion neuron loss in a complex 1 knockout mouse model.
Mitochondrial Diseases
Biventricular non-compaction hypertrophic cardiomyopathy in association with congenital complete heart block and type I mitochondrial complex deficiency.
Mitochondrial Diseases
Bromodomain Inhibitors Correct Bioenergetic Deficiency Caused by Mitochondrial Disease Complex I Mutations.
Mitochondrial Diseases
Building a complex complex: Assembly of mitochondrial respiratory chain complex I.
Mitochondrial Diseases
Caenorhabditis elegans expressing the Saccharomyces cerevisiae NADH alternative dehydrogenase Ndi1p, as a tool to identify new genes involved in complex I related diseases.
Mitochondrial Diseases
Can a single subunit yeast NADH dehydrogenase (Ndi1) remedy diseases caused by respiratory complex I defects?
Mitochondrial Diseases
Cardiomyopathy and angiopathy in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes.
Mitochondrial Diseases
Case Report: SATB2-Associated Syndrome Overlapping With Clinical Mitochondrial Disease Presentation: Report of Two Cases.
Mitochondrial Diseases
Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency.
Mitochondrial Diseases
Cerebellar ataxia in patients with mitochondrial DNA disease: a molecular clinicopathological study.
Mitochondrial Diseases
Chapter 7 Tracing human mitochondrial complex I assembly by use of GFP-tagged subunits.
Mitochondrial Diseases
Characterization of clinically identified mutations in NDUFV1, the flavin-binding subunit of respiratory complex I, using a yeast model system.
Mitochondrial Diseases
Cigarette toxicity triggers Leber's hereditary optic neuropathy by affecting mtDNA copy number, oxidative phosphorylation and ROS detoxification pathways.
Mitochondrial Diseases
Clinical, biochemical, and genetic analysis of the mitochondrial respiratory chain complex I deficiency.
Mitochondrial Diseases
Coenzyme Q cytoprotective mechanisms for mitochondrial complex I cytopathies involves NAD(P)H: quinone oxidoreductase 1(NQO1).
Mitochondrial Diseases
Complex I deficiency: clinical features, biochemistry and molecular genetics.
Mitochondrial Diseases
Complex I impairment in mitochondrial diseases and cancer: Parallel roads leading to different outcomes.
Mitochondrial Diseases
Cybrid studies establish the causal link between the mtDNA m.3890G>A/MT-ND1 mutation and optic atrophy with bilateral brainstem lesions.
Mitochondrial Diseases
Cytoplasmic transfer of platelet mtDNA from elderly patients with Parkinson's disease to mtDNA-less HeLa cells restores complete mitochondrial respiratory function.
Mitochondrial Diseases
De novo mutations in the mitochondrial ND3 gene as a cause of infantile mitochondrial encephalopathy and complex I deficiency.
Mitochondrial Diseases
Decoding mitochondrial heterogeneity in single muscle fibres by imaging mass cytometry.
Mitochondrial Diseases
Deep intronic TIMMDC1 variant delays diagnosis of rapidly progressive complex I deficiency.
Mitochondrial Diseases
Defective NADPH production in mitochondrial disease complex I causes inflammation and cell death.
Mitochondrial Diseases
Deletions of mitochondrial DNA in Kearns-Sayre syndrome and ocular myopathies: genetic, biochemical and morphological studies.
Mitochondrial Diseases
Diverse phenotype in patients with complex I deficiency due to mutations in NDUFB11.
Mitochondrial Diseases
DNAJC30 biallelic mutations extend mitochondrial complex I-deficient phenotypes to include recessive Leber's hereditary optic neuropathy.
Mitochondrial Diseases
Dopamine transporter SPECT in patients with mitochondrial disorders.
Mitochondrial Diseases
Early cardiac evaluation in children with non-specific mitochondrial disease with isolated mitochondrial respiratory chain complex I defect.
Mitochondrial Diseases
Effects of disrupting the 21 kDa subunit of complex I from Neurospora crassa.
Mitochondrial Diseases
Endotracheal Intubation Using Alfentanil in a Pediatric Patient with a Mitochondrial Myopathy and Gastroparesis.
Mitochondrial Diseases
ER and Nutrient Stress Promote Assembly of Respiratory Chain Supercomplexes through the PERK-eIF2? Axis.
Mitochondrial Diseases
Evaluating clinical mitochondrial respiratory chain enzymes from biopsy specimens presenting skewed probability distribution of activity data.
Mitochondrial Diseases
Exome sequencing identifies ACAD9 mutations as a cause of complex I deficiency.
Mitochondrial Diseases
Expression of Ndi1p, an alternative NADH:ubiquinone oxidoreductase, increases mitochondrial membrane potential in a C. elegans model of mitochondrial disease.
Mitochondrial Diseases
Fatal infantile mitochondrial encephalomyopathy with complex I and IV deficiencies.
Mitochondrial Diseases
FOXRED1, encoding an FAD-dependent oxidoreductase complex-I-specific molecular chaperone, is mutated in infantile-onset mitochondrial encephalopathy.
Mitochondrial Diseases
Generation of patient-specific induced pluripotent stem cells from Leber's hereditary optic neuropathy.
Mitochondrial Diseases
Generation of trans-mitochondrial mito-mice by the introduction of a pathogenic G13997A mtDNA from highly metastatic lung carcinoma cells.
Mitochondrial Diseases
Genome sequencing and RNA-seq analyses of mitochondrial complex I deficiency revealed Alu insertion-mediated deletion in NDUFV2.
Mitochondrial Diseases
Genotyping microsatellite DNA markers at putative disease loci in inbred/multiplex families with respiratory chain complex I deficiency allows rapid identification of a novel nonsense mutation (IVS1nt -1) in the NDUFS4 gene in Leigh syndrome.
Mitochondrial Diseases
Glial lipid droplets and neurodegeneration in a Drosophila model of complex I deficiency.
Mitochondrial Diseases
Glucose Transporter Type I Deficiency Causing Mitochondrial Dysfunction.
Mitochondrial Diseases
Hepatic S6K1 Partially Regulates Lifespan of Mice with Mitochondrial Complex I Deficiency.
Mitochondrial Diseases
Human CIA30 is involved in the early assembly of mitochondrial complex I and mutations in its gene cause disease.
Mitochondrial Diseases
Human Ind1, an iron-sulfur cluster assembly factor for respiratory complex I.
Mitochondrial Diseases
Human mitochondrial complex I assembles through the combination of evolutionary conserved modules: a framework to interpret complex I deficiencies.
Mitochondrial Diseases
Identification and characterization of a common set of complex I assembly intermediates in mitochondria from patients with complex I deficiency.
Mitochondrial Diseases
Identification and Characterization of New Variants in FOXRED1 Gene Expands the Clinical Spectrum Associated with Mitochondrial Complex I Deficiency.
Mitochondrial Diseases
Impaired hypoxic pulmonary vasoconstriction in a mouse model of Leigh syndrome.
Mitochondrial Diseases
Insights into the pathogenic character of a common NUBPL branch-site mutation associated with mitochondrial disease and complex I deficiency using a yeast model.
Mitochondrial Diseases
Introduction of an additional pathway for lactate oxidation in the treatment of lactic acidosis and mitochondrial dysfunction in Caenorhabditis elegans.
Mitochondrial Diseases
Investigating complex I deficiency in Purkinje cells and synapses in patients with mitochondrial disease.
Mitochondrial Diseases
Isolated and repeated stroke-like episodes in a middle-aged man with a mitochondrial ND3 T10158C mutation: a case report.
Mitochondrial Diseases
Lack of complex I activity in human cells carrying a mutation in MtDNA-encoded ND4 subunit is corrected by the Saccharomyces cerevisiae NADH-quinone oxidoreductase (NDI1) gene.
Mitochondrial Diseases
Late-Onset Leigh Syndrome due to NDUFV1 Mutation in a 10-Year-Old Boy Initially Presenting with Ataxia.
Mitochondrial Diseases
Lethal infantile mitochondrial disease with isolated complex I deficiency in fibroblasts but with combined complex I and IV deficiencies in muscle.
Mitochondrial Diseases
Mechanism of action and potential applications of selective inhibition of microsomal prostaglandin E synthase-1-mediated PGE2 biosynthesis by sonlicromanol's metabolite KH176m.
Mitochondrial Diseases
Metabolome and proteome profiling of complex I deficiency induced by rotenone.
Mitochondrial Diseases
Metformin delays neurological symptom onset in a mouse model of neuronal complex I deficiency.
Mitochondrial Diseases
Mitochondria as the target for mildronate's protective effects in azidothymidine (AZT)-induced toxicity of isolated rat liver mitochondria.
Mitochondrial Diseases
Mitochondrial Complex I Mutations Predispose Drosophila to Isoflurane Neurotoxicity.
Mitochondrial Diseases
Mitochondrial complex II prevents hypoxic but not calcium- and proapoptotic Bcl-2 protein-induced mitochondrial membrane potential loss.
Mitochondrial Diseases
Mitochondrial disease associated with complex I (NADH-CoQ oxidoreductase) deficiency.
Mitochondrial Diseases
Mitochondrial DNA background modulates the assembly kinetics of OXPHOS complexes in a cellular model of mitochondrial disease.
Mitochondrial Diseases
Mitochondrial dynamics in human NADH:ubiquinone oxidoreductase deficiency.
Mitochondrial Diseases
Mitochondrial enzymes discriminate between mitochondrial disorders and chronic fatigue syndrome.
Mitochondrial Diseases
Mitochondrial respiratory chain dysfunction in various neuromuscular diseases.
Mitochondrial Diseases
Mitophagy activation repairs Leber's hereditary optic neuropathy-associated mitochondrial dysfunction and improves cell survival.
Mitochondrial Diseases
Molecular defects of NADH-ubiquinone oxidoreductase (complex I) in mitochondrial diseases.
Mitochondrial Diseases
Molecular remedy of complex I defects: rotenone-insensitive internal NADH-quinone oxidoreductase of Saccharomyces cerevisiae mitochondria restores the NADH oxidase activity of complex I-deficient mammalian cells.
Mitochondrial Diseases
Mouse studies to shape clinical trials for mitochondrial diseases: high fat diet in Harlequin mice.
Mitochondrial Diseases
Multisystem mitochondrial diseases due to mutations in mtDNA-encoded subunits of complex I.
Mitochondrial Diseases
Mutation of c20orf7 disrupts complex I assembly and causes lethal neonatal mitochondrial disease.
Mitochondrial Diseases
Mutation screening of 75 candidate genes in 152 complex I deficiency cases identifies pathogenic variants in 16 genes including NDUFB9.
Mitochondrial Diseases
Mutations in human nuclear genes encoding for subunits of mitochondrial respiratory complex I: the NDUFS4 gene.
Mitochondrial Diseases
Mutations in ND subunits of complex I are an important genetic cause of childhood mitochondrial encephalopathies.
Mitochondrial Diseases
Mutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex I assembly protein, cause fatal neonatal mitochondrial disease.
Mitochondrial Diseases
Mutations in the mitochondrial complex I assembly factor NDUFAF1 cause fatal infantile hypertrophic cardiomyopathy.
Mitochondrial Diseases
Ndufaf5 deficiency in the Dictyostelium model: new roles in autophagy and development.
Mitochondrial Diseases
NDUFB8 Mutations Cause Mitochondrial Complex I Deficiency in Individuals with Leigh-like Encephalomyopathy.
Mitochondrial Diseases
NDUFS3 depletion permits complex I maturation and reveals TMEM126A/OPA7 as an assembly factor binding the ND4-module intermediate.
Mitochondrial Diseases
Neurospora strains harboring mitochondrial disease-associated mutations in iron-sulfur subunits of complex I.
Mitochondrial Diseases
Nitric oxide, complex I, and the modulation of mitochondrial reactive species in biology and disease.
Mitochondrial Diseases
Normal muscle respiratory chain enzymes can complicate mitochondrial disease diagnosis.
Mitochondrial Diseases
Novel MT-ND Gene Variants Causing Adult-Onset Mitochondrial Disease and Isolated Complex I Deficiency.
Mitochondrial Diseases
Novel mutations of ND genes in complex I deficiency associated with mitochondrial encephalopathy.
Mitochondrial Diseases
Oestrogens ameliorate mitochondrial dysfunction in Leber's hereditary optic neuropathy.
Mitochondrial Diseases
Optic neuropathy linked to ACAD9 pathogenic variants: A potentially riboflavin-responsive disorder?
Mitochondrial Diseases
Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage.
Mitochondrial Diseases
Pathogenic mutations in NUBPL affect complex I activity and cold tolerance in the yeast model Yarrowia lipolytica.
Mitochondrial Diseases
Pathological mechanisms underlying single large-scale mitochondrial DNA deletions.
Mitochondrial Diseases
Photoreceptors in a mouse model of Leigh syndrome are capable of normal light-evoked signaling.
Mitochondrial Diseases
Polarographic evaluation of mitochondrial enzymes activity in isolated mitochondria and in permeabilized human muscle cells with inherited mitochondrial defects.
Mitochondrial Diseases
Predominant cerebellar volume loss as a neuroradiologic feature of pediatric respiratory chain defects.
Mitochondrial Diseases
Prerequisites and strategies for prenatal diagnosis of respiratory chain deficiency in chorionic villi.
Mitochondrial Diseases
Recurrent acute liver failure and mitochondriopathy in a case of Wolcott-Rallison syndrome.
Mitochondrial Diseases
Region-specific expression of mitochondrial complex I genes during murine brain development.
Mitochondrial Diseases
Replacement of the C6ORF66 Assembly Factor (NDUFAF4) Restores Complex I Activity in Patient Cells.
Mitochondrial Diseases
Respiratory chain complex I deficiency caused by mitochondrial DNA mutations.
Mitochondrial Diseases
Resveratrol attenuates oxidative stress in mitochondrial Complex I deficiency: Involvement of SIRT3.
Mitochondrial Diseases
Riboflavin enhances the assembly of mitochondrial cytochrome c oxidase in C. elegans NADH-ubiquinone oxidoreductase mutants.
Mitochondrial Diseases
Semiquinone intermediates are involved in the energy coupling mechanism of E. coli complex I.
Mitochondrial Diseases
Serine residues at positions 162 and 166 of the rabies virus phosphoprotein are critical for the induction of oxidative stress in rabies virus infection.
Mitochondrial Diseases
Species-specific and mutant MWFE proteins. Their effect on the assembly of a functional mammalian mitochondrial complex I.
Mitochondrial Diseases
Spectrophotometric assay for complex I of the respiratory chain in tissue samples and cultured fibroblasts.
Mitochondrial Diseases
Spondyloepimetaphyseal dysplasia with neurodegeneration associated with AIFM1 mutation - a novel phenotype of the mitochondrial disease.
Mitochondrial Diseases
Subcomplex Ilambda specifically controls integrated mitochondrial functions in Caenorhabditis elegans.
Mitochondrial Diseases
Subunits of mitochondrial complex I exist as part of matrix- and membrane-associated subcomplexes in living cells.
Mitochondrial Diseases
The addition of ketone bodies alleviates mitochondrial dysfunction by restoring complex I assembly in a MELAS cellular model.
Mitochondrial Diseases
The Effect of Mitochondrial Supplements on Mitochondrial Activity in Children with Autism Spectrum Disorder.
Mitochondrial Diseases
The mutant human ND4 subunit of complex I induces optic neuropathy in the mouse.
Mitochondrial Diseases
The NDUFS4 Knockout Mouse: A Dual Threat Model of Childhood Mitochondrial Disease and Normative Aging.
Mitochondrial Diseases
The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families.
Mitochondrial Diseases
The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification.
Mitochondrial Diseases
Therapeutic applications of the TAT-mediated protein transduction system for complex I deficiency and other mitochondrial diseases.
Mitochondrial Diseases
Titrating the effects of mitochondrial complex I impairment in the cell physiology.
Mitochondrial Diseases
Tract-Based Spatial Statistical Analysis of Diffusion Tensor Imaging in Pediatric Patients with Mitochondrial Disease: Widespread Reduction in Fractional Anisotropy of White Matter Tracts.
Mitochondrial Diseases
Understanding mitochondrial complex I assembly in health and disease.
Mitochondrial Diseases
Uniparental isodisomy as a cause of mitochondrial complex I respiratory chain disorder due to a novel splicing NDUFS4 mutation.
Mitochondrial Diseases
Update on clinical aspects and treatment of selected vitamin-responsive disorders II (riboflavin and CoQ(10)).
Mitochondrial Diseases
Use of the Ketogenic Diet to Treat Intractable Epilepsy in Mitochondrial Disorders.
Mitochondrial Diseases
Use of the NADH-quinone oxidoreductase (NDI1) gene of Saccharomyces cerevisiae as a possible cure for complex I defects in human cells.
Mitochondrial Diseases
Visual function, ocular motility and ocular characteristics in patients with mitochondrial complex I deficiency.
Mitochondrial Diseases
[High resolution respirometry in diagnostic of mitochondrial disorders caused by mitochondrial complex I deficiency].
Mitochondrial Diseases
[Release of flavin from the mitochondrial NADH-dehydrogenase complex]
Mitochondrial Encephalomyopathies
A mitochondrial encephalomyopathy with cardiomyopathy. A case revealing a defect of complex I in the respiratory chain.
Mitochondrial Encephalomyopathies
A mitochondrial encephalomyopathy with specific deficiencies of two respiratory chain polypeptides and a circulating autoantibody to a mitochondrial matrix protein.
Mitochondrial Encephalomyopathies
A slowly progressive mitochondrial encephalomyopathy widens the spectrum of AIFM1 disorders.
Mitochondrial Encephalomyopathies
An autopsy case of mitochondrial encephalomyopathy with prominent degeneration in olivo-ponto-cerebellar system.
Mitochondrial Encephalomyopathies
Association of the mtDNA m.4171C>A/MT-ND1 mutation with both optic neuropathy and bilateral brainstem lesions.
Mitochondrial Encephalomyopathies
Benign mitochondrial encephalomyopathy in a patient with complex I deficiency.
Mitochondrial Encephalomyopathies
C6ORF66 is an assembly factor of mitochondrial complex I.
Mitochondrial Encephalomyopathies
Changing phenotypic expression in a patient with a mitochondrial encephalopathy due to 13042G>A de novo mutation-a 5 year follow up.
Mitochondrial Encephalomyopathies
Characterization of the human complex I NDUFB7 and 17.2-kDa cDNAs and mutational analysis of 19 genes of the HP fraction in complex I-deficient-patients.
Mitochondrial Encephalomyopathies
Clinical and molecular heterogeneity in patients with mitochondrial encephalomyopathies due to complex I deficiency.
Mitochondrial Encephalomyopathies
Complex I spectrophotometric assay in cultured cells: detailed analysis of key factors.
Mitochondrial Encephalomyopathies
Cowchock syndrome is associated with a mutation in apoptosis-inducing factor.
Mitochondrial Encephalomyopathies
Deficiency of subunits in heart mitochondrial NADH-ubiquinone oxidoreductase of a patient with mitochondrial encephalomyopathy and cardiomyopathy.
Mitochondrial Encephalomyopathies
Deficiency of subunits of Complex I and mitochondrial encephalomyopathy.
Mitochondrial Encephalomyopathies
Development and characterization of polyspecific anti-mitochondrion antibodies for proteomics studies on in toto tissue homogenates.
Mitochondrial Encephalomyopathies
Disease-causing mitochondrial heteroplasmy segregated within induced pluripotent stem cell clones derived from a patient with MELAS.
Mitochondrial Encephalomyopathies
Disproportionate deficiency of iron-sulfur clusters and subunits of complex I in mitochondrial encephalomyopathy.
Mitochondrial Encephalomyopathies
Dysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiency.
Mitochondrial Encephalomyopathies
Fatal infantile mitochondrial encephalomyopathy with complex I and IV deficiencies.
Mitochondrial Encephalomyopathies
High resolution respirometry of permeabilized skeletal muscle fibers in the diagnosis of neuromuscular disorders.
Mitochondrial Encephalomyopathies
Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes with special reference to the mechanism of cerebral manifestations.
Mitochondrial Encephalomyopathies
Mitochondrial encephalomyopathy. Association with an NADH dehydrogenase deficiency.
Mitochondrial Encephalomyopathies
Mitochondrial Encephalopathy and Optic Neuropathy Due to m.10158 MT-ND3 Complex I Mutation Presenting in an Adult Patient: Case Report and Review of the Literature.
Mitochondrial Encephalomyopathies
Mitochondrial variations in the MT-ND4 and MT-TL1 genes are associated with male infertility.
Mitochondrial Encephalomyopathies
Mutations in AIFM1 cause an X-linked childhood cerebellar ataxia partially responsive to riboflavin.
Mitochondrial Encephalomyopathies
Novel mitochondrial DNA ND5 mutation in a patient with clinical features of MELAS and MERRF.
Mitochondrial Encephalomyopathies
Pathogenic SLIRP variants as a novel cause of autosomal recessive mitochondrial encephalomyopathy with complex I and IV deficiency.
Mitochondrial Encephalomyopathies
Predominant cerebellar volume loss as a neuroradiologic feature of pediatric respiratory chain defects.
Mitochondrial Encephalomyopathies
The addition of ketone bodies alleviates mitochondrial dysfunction by restoring complex I assembly in a MELAS cellular model.
Mitochondrial Encephalomyopathies
The m.3890G>A/MT-ND1 mtDNA rare pathogenic variant: Expanding clinical and MRI phenotypes.
Mitochondrial Encephalomyopathies
The mitochondrial 13513G > A mutation is most frequent in Leigh syndrome combined with reduced complex I activity, optic atrophy and/or Wolff-Parkinson-White.
Mitochondrial Encephalomyopathies
The role of complex I genes in MELAS: a novel heteroplasmic mutation 3380G>A in ND1 of mtDNA.
Mitochondrial Encephalomyopathies
The X-chromosomal NDUFA1 gene of complex I in mitochondrial encephalomyopathies: tissue expression and mutation detection.
Mitochondrial Encephalomyopathies
Transition from Leigh syndrome to MELAS syndrome in a patient with heteroplasmic MT-ND3 m.10158T>C.
Mitochondrial Encephalomyopathies
Variation in the levels of complex I subunits among tissues in a patient with mitochondrial encephalomyopathy and renal dysfunction.
Mitochondrial Encephalomyopathies
X-linked NDUFA1 gene mutations associated with mitochondrial encephalomyopathy.
Mitochondrial Encephalomyopathies
[A case of mitochondrial encephalomyopathy with a defect in electron transport at complex I and IV in skeletal muscle showing peripheral neuropathy]
Mitochondrial Encephalomyopathies
[A case of mitochondrial encephalomyopathy with cardiomyopathy due to decreased complex I and IV activities]
Mitochondrial Encephalomyopathies
[A mitochondrial encephalomyopathy: a case of a defect of complex I in the electron transport chain]
Mitochondrial Encephalomyopathies
[Complex I (NADH coenzyme-Q-reductase) deficiency, MELAS syndrome and hypertrophic cardiomyopathy]
Mitochondrial Myopathies
A 31P magnetic resonance spectroscopy study of mitochondrial function in skeletal muscle of patients with Parkinson's disease.
Mitochondrial Myopathies
A case of mitochondrial myopathy, lactic acidosis and complex I deficiency.
Mitochondrial Myopathies
A mitochondrial myopathy with a deficiency of respiratory chain NADH-CoQ reductase activity.
Mitochondrial Myopathies
A novel mechanism causing imbalance of mitochondrial fusion and fission in human myopathies.
Mitochondrial Myopathies
A novel mutation in the mitochondrial tRNA(Ser(AGY)) gene associated with mitochondrial myopathy, encephalopathy, and complex I deficiency.
Mitochondrial Myopathies
Abnormal RNA processing associated with a novel tRNA mutation in mitochondrial DNA. A potential disease mechanism.
Mitochondrial Myopathies
An animal model of mitochondrial myopathy: a biochemical and physiological investigation of rats treated in vivo with the NADH-CoQ reductase inhibitor, diphenyleneiodonium.
Mitochondrial Myopathies
Benign mitochondrial myopathy with deficiency of NADH-CoQ reductase and cytochrome c oxidase.
Mitochondrial Myopathies
Deficiency of subunits of Complex I and mitochondrial encephalomyopathy.
Mitochondrial Myopathies
Deficiency of subunits of complex I or IV in mitochondrial myopathies: immunochemical and immunohistochemical study.
Mitochondrial Myopathies
Deletions of muscle mitochondrial DNA in patients with mitochondrial myopathies.
Mitochondrial Myopathies
Disproportionate deficiency of iron-sulfur clusters and subunits of complex I in mitochondrial encephalomyopathy.
Mitochondrial Myopathies
Exercise intolerance due to a nonsense mutation in the mtDNA ND4 gene.
Mitochondrial Myopathies
Expanding the phenotype of de novo SLC25A4-linked mitochondrial disease to include mild myopathy.
Mitochondrial Myopathies
Familial mitochondrial myopathy associated with peripheral neuropathy: partial deficiencies of complex I and complex IV.
Mitochondrial Myopathies
Fatal mitochondrial myopathy, lactic acidosis, and complex I deficiency associated with a heteroplasmic A --> G mutation at position 3251 in the mitochondrial tRNALeu(UUR) gne.
Mitochondrial Myopathies
In vitro analysis of rod composition and actin dynamics in inherited myopathies.
Mitochondrial Myopathies
Increasing fat in the diet does not improve muscle performance in patients with mitochondrial myopathy due to complex I deficiency.
Mitochondrial Myopathies
Intragenic inversion of mtDNA: a new type of pathogenic mutation in a patient with mitochondrial myopathy.
Mitochondrial Myopathies
Isolated and combined deficiencies of NADH dehydrogenase (complex I) in muscle tissue of children with mitochondrial myopathies.
Mitochondrial Myopathies
Lactate disposal via gluconeogenesis is increased during exercise in patients with mitochondrial myopathy due to complex I deficiency.
Mitochondrial Myopathies
LHON/MELAS overlap mutation in ND1 subunit of mitochondrial complex I affects ubiquinone binding as revealed by modeling in Escherichia coli NDH-1.
Mitochondrial Myopathies
Mice Lacking TR4 Nuclear Receptor Develop Mitochondrial Myopathy with Deficiency in Complex I.
Mitochondrial Myopathies
Mitochondrial DNA mutations in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS).
Mitochondrial Myopathies
Mitochondrial myopathies: deficiencies localized to complex I and complex III of the mitochondrial respiratory chain.
Mitochondrial Myopathies
Mitochondrial myopathy with tRNA(Leu(UUR)) mutation and complex I deficiency responsive to riboflavin.
Mitochondrial Myopathies
Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes syndrome and NADH-CoQ reductase deficiency.
Mitochondrial Myopathies
Mitochondrial NADH-ubiquinone reductase: complementary DNA sequences of import precursors of the bovine and human 24-kDa subunit.
Mitochondrial Myopathies
Molecular cloning of the rat NADH:ubiquinone oxidoreductase subunit and its up-regulation in the facial muscle after denervation: detected by means of differential display.
Mitochondrial Myopathies
Multiple deficiencies of mitochondrial DNA- and nuclear-encoded subunits of respiratory NADH dehydrogenase detected with peptide- and subunit-specific antibodies in mitochondrial myopathies.
Mitochondrial Myopathies
Mutations in mitochondrially encoded complex I enzyme as the second common cause in a cohort of Chinese patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes.
Mitochondrial Myopathies
Myopathy reversion in mice after restauration of mitochondrial complex I.
Mitochondrial Myopathies
Partial deficiency of subunits in complex I or IV of patients with mitochondrial myopathies.
Mitochondrial Myopathies
Triacylglycerol infusion does not improve hyperlactemia in resting patients with mitochondrial myopathy due to complex I deficiency.
Mitochondrial Myopathies
Triacylglycerol infusion improves exercise endurance in patients with mitochondrial myopathy due to complex I deficiency.
Motor Neuron Disease
A novel AIFM1 mutation expands the phenotype to an infantile motor neuron disease.
Motor Neuron Disease
Inhibition of cystathionine-gamma-lyase leads to loss of glutathione and aggravation of mitochondrial dysfunction mediated by excitatory amino acid in the CNS.
Mouth Neoplasms
Magnolia extract is effective for the chemoprevention of oral cancer through its ability to inhibit mitochondrial respiration at complex I.
Movement Disorders
Dementia in Parkinson's disease is associated with enhanced mitochondrial complex I deficiency.
MPTP Poisoning
Is there a special relationship between complex I activity and nigral neuronal loss in Parkinson's disease? A critical reappraisal.
Multiple Myeloma
MIR145-3p promotes autophagy and enhances bortezomib sensitivity in multiple myeloma by targeting HDAC4.
Multiple Myeloma
Synthesis and SAR of Lehualide B: a marine-derived natural product with potent anti-multiple myeloma activity.
Multiple Myeloma
Targeting MUC1-C suppresses polycomb repressive complex 1 in multiple myeloma.
Multiple Myeloma
The PP242 mammalian target of rapamycin (mTOR) inhibitor activates extracellular signal-regulated kinase (ERK) in multiple myeloma cells via a target of rapamycin complex 1 (TORC1)/eukaryotic translation initiation factor 4E (eIF-4E)/RAF pathway and activation is a mechanism of resistance.
Multiple Sclerosis
A novel mitochondrial DNA deletion producing progressive external ophthalmoplegia associated with multiple sclerosis.
Multiple Sclerosis
Four novel mutations in the mitochondrial ND4 gene of complex I in patients with multiple sclerosis.
Multiple Sclerosis
Gene Therapy with Single-Subunit Yeast NADH-Ubiquinone Oxidoreductase (NDI1) Improves the Visual Function in Experimental Autoimmune Encephalomyelitis (EAE) Mice Model of Multiple Sclerosis (MS).
Multiple Sclerosis
GRIM-19 Ameliorates Multiple Sclerosis in a Mouse Model of Experimental Autoimmune Encephalomyelitis with Reciprocal Regulation of IFN?/Th1 and IL-17A/Th17 Cells.
Multiple Sclerosis
Mitochondrial complex I gene variations; as a potential genetic risk factor in pathogenesis of multiple sclerosis.
Multiple Sclerosis
NADH-dehydrogenase Type-2 Suppresses Irreversible Visual Loss and Neurodegeneration in the EAE Animal Model of MS.
Multiple Sclerosis
Naturally presented peptides on major histocompatibility complex I and II molecules eluted from central nervous system of multiple sclerosis patients.
Multiple Sclerosis
Phytocannabinoid-dependent mTORC1 regulation is dependent upon inositol polyphosphate multikinase activity.
Multiple System Atrophy
Mitochondrial function in Parkinson's disease. The Royal Kings and Queens Parkinson's Disease Research Group.
Muscle Hypotonia
Clinical presentation of mitochondrial respiratory chain defects in NADH-coenzyme Q reductase and cytochrome oxidase: clues to pathogenesis of Leigh disease.
Muscle Hypotonia
Mutant NDUFV2 subunit of mitochondrial complex I causes early onset hypertrophic cardiomyopathy and encephalopathy.
Muscle Hypotonia
Mutation in NDUFA13/GRIM19 leads to early onset hypotonia, dyskinesia and sensorial deficiencies, and mitochondrial complex I instability.
Muscle Hypotonia
Nemaline rods and complex I deficiency in three infants with hypotonia, motor delay and failure to thrive.
Muscle Hypotonia
VARS2 and TARS2 mutations in patients with mitochondrial encephalomyopathies.
Muscle Spasticity
Genetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystonia.
Muscle Weakness
A novel mutation in the human complex I NDUFS7 subunit associated with Leigh syndrome.
Muscle Weakness
Multiple defects of the respiratory chain including complex II in a family with myopathy and encephalopathy.
Muscle Weakness
Mutations in Complex I Assembly Factor TMEM126B Result in Muscle Weakness and Isolated Complex I Deficiency.
Muscle Weakness
Sequence analysis of nuclear genes encoding functionally important complex I subunits in children with encephalomyopathy.
Muscle Weakness
Successful treatment of infantile-onset ACAD9-related cardiomyopathy with a combination of sodium pyruvate, beta-blocker, and coenzyme Q10.
Muscular Atrophy
Daily protein supplementation attenuates immobilization-induced blunting of postabsorptive muscle mTORC1 activation in middle-aged men.
Muscular Atrophy
From ventriculomegaly to severe muscular atrophy: Expansion of the clinical spectrum related to mutations in AIFM1.
Muscular Atrophy
Growth hormone and Insulin-like growth factor-I (IGF-I) modulate the expression of L-type amino acid transporters in the muscles of spontaneous dwarf rats and L6 and C2C12 myocytes.
Muscular Atrophy
Insulin prevents and reverts simvastatin-induced toxicity in C2C12 skeletal muscle cells.
Muscular Atrophy
Protein Supplementation Enhances the Effects of Intermittent Loading on Skeletal Muscles by Activating the mTORC1 Signaling Pathway in a Rat Model of Disuse Atrophy.
Muscular Atrophy
The analysis of antioxidant expression during muscle atrophy induced by hindlimb suspension in mice.
Muscular Atrophy, Spinal
Mitochondrial respiratory complex I deficiency simulating spinal muscular atrophy.
Muscular Atrophy, Spinal
SETX (senataxin), the helicase mutated in AOA2 and ALS4, functions in autophagy regulation.
Muscular Diseases
A disease-associated Aifm1 variant induces severe myopathy in knockin mice.
Muscular Diseases
A heteroplasmic mitochondrial complex I gene mutation in adult-onset dystonia.
Muscular Diseases
A mitochondrial encephalomyopathy with cardiomyopathy. A case revealing a defect of complex I in the respiratory chain.
Muscular Diseases
A novel mitochondrial DNA deletion producing progressive external ophthalmoplegia associated with multiple sclerosis.
Muscular Diseases
A novel mitochondrial MTND5 frameshift mutation causing isolated complex I deficiency, renal failure and myopathy.
Muscular Diseases
Analysis of mitochondrial DNA sequences in patients with isolated or combined oxidative phosphorylation system deficiency.
Muscular Diseases
Biallelic Mutations in TMEM126B Cause Severe Complex I Deficiency with a Variable Clinical Phenotype.
Muscular Diseases
Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease.
Muscular Diseases
Complex I (reduced nicotinamide-adenine dinucleotide-coenzyme Q reductase) deficiency in two patients with probable Leigh syndrome.
Muscular Diseases
Congenital ocular motor apraxia associated with myopathy, external hydrocephalus and NADH dehydrogenase deficiency.
Muscular Diseases
Deficiency of the reduced nicotinamide adenine dinucleotide dehydrogenase component of complex I of mitochondrial electron transport. Fatal infantile lactic acidosis and hypermetabolism with skeletal-cardiac myopathy and encephalopathy.
Muscular Diseases
From ventriculomegaly to severe muscular atrophy: Expansion of the clinical spectrum related to mutations in AIFM1.
Muscular Diseases
Genetic diversity of NDUFV1-dependent mitochondrial complex I deficiency.
Muscular Diseases
Immunohistochemical analysis of the oxidative phosphorylation complexes in skeletal muscle from patients with mitochondrial DNA encoded tRNA gene defects.
Muscular Diseases
Integrated proteomic and transcriptomic profiling identifies aberrant gene and protein expression in the sarcomere, mitochondrial complex I, and the extracellular matrix in Warmblood horses with myofibrillar myopathy.
Muscular Diseases
Mitochondrial complex I and III mutations and neutral-lipid storage in activated mononuclear macrophages and neutrophils: a case presenting with necrotizing myopathy, poikiloderma atrophicans vasculare, and xanthogranulomatous bursitis.
Muscular Diseases
Mitochondrial myopathy with tRNA(Leu(UUR)) mutation and complex I deficiency responsive to riboflavin.
Muscular Diseases
Mitochondrial tRNA(Leu(UUR)) mutation m.3302A > G presenting as childhood-onset severe myopathy: threshold determination through segregation study.
Muscular Diseases
Multiple deficiencies of mitochondrial DNA- and nuclear-encoded subunits of respiratory NADH dehydrogenase detected with peptide- and subunit-specific antibodies in mitochondrial myopathies.
Muscular Diseases
Myopathy caused by mammalian target of rapamycin complex 1 (mTORC1) inactivation is not reversed by restoring mitochondrial function.
Muscular Diseases
Myopathy reversion in mice after restauration of mitochondrial complex I.
Muscular Diseases
NADH-CoQ reductase deficient myopathy: successful treatment with riboflavin.
Muscular Diseases
Novel mutations in KARS cause hypertrophic cardiomyopathy and combined mitochondrial respiratory chain defect.
Muscular Diseases
Quantitative analysis of proteins of metabolism by reverse phase protein microarrays identifies potential biomarkers of rare neuromuscular diseases.
Muscular Diseases
Resting oxygen consumption and in vivo ADP are increased in myopathy due to complex I deficiency.
Muscular Diseases
Severe defect in mitochondrial complex I assembly with mtDNA deletions in ACAD9-deficient mild myopathy.
Muscular Diseases
Successful treatment of pure myopathy, associated with complex I deficiency, with riboflavin and carnitine.
Muscular Diseases
The expanding LARS2 phenotypic spectrum: HLASA, Perrault syndrome with leukodystrophy, and mitochondrial myopathy.
Muscular Diseases
[Mitochondrial encephalopathies with late disclosure and predominant involvement of central nervous system]
Muscular Disorders, Atrophic
The mTORC1 signaling repressors REDD1/2 are rapidly induced and activation of p70S6K1 by leucine is defective in skeletal muscle of an immobilized rat hindlimb.
Muscular Dystrophies
Polycomb repressive complex 1 provides a molecular explanation for repeat copy number dependency in FSHD muscular dystrophy.
Muscular Dystrophies
Quantitative analysis of proteins of metabolism by reverse phase protein microarrays identifies potential biomarkers of rare neuromuscular diseases.
Muscular Dystrophies, Limb-Girdle
Quantitative analysis of proteins of metabolism by reverse phase protein microarrays identifies potential biomarkers of rare neuromuscular diseases.
Muscular Dystrophy, Duchenne
Impairments in left ventricular mitochondrial bioenergetics precede overt cardiac dysfunction and remodelling in Duchenne muscular dystrophy.
Myelodysplastic Syndromes
The role of autophagy in targeted therapy for acute myeloid leukemia.
Myocardial Infarction
Mitochondrial autophagy and cell survival is regulated by the circadian Clock gene in cardiac myocytes during ischemic stress.
Myocardial Infarction
Relationship between energy liberation and utilization in ischemic cardiac muscle.
Myocardial Infarction
Sub-endocardial increase in ROS production affects regional contractile function in ischemic heart failure.
Myocardial Infarction
VEGF-B-induced vascular growth leads to metabolic reprogramming and ischemia resistance in the heart.
Myocardial Ischemia
Canine myocardial ischemia: defect in mitochondrial electron transfer complex I.
Myocardial Ischemia
Chronic Myocardial Ischemia Leads to Loss of Maximal Oxygen Consumption and Complex I Dysfunction.
Myocardial Ischemia
Effects of acidosis and ATP depletion on cardiac muscle electron transfer complex I.
Myocardial Ischemia
Impaired function of mitochondrial electron transfer complex I in canine myocardial ischemia: loss of flavin mononucleotide.
Myocardial Ischemia
Interaction between longevity-associated mitochondrial DNA 5178 C/A polymorphism and cigarette smoking on hematological parameters in Japanese men.
Myocardial Ischemia
Mitochondrial inner membrane enzyme defects in porcine myocardial ischemia.
Myocardial Ischemia
Mitochondrial respiratory chain enzyme activities in tetralogy of Fallot.
Myocardial Ischemia
Substrate-specific impairment of mitochondrial respiration in permeabilized fibers from patients with coronary heart disease versus valvular disease.
Myocardial Stunning
New perspectives of nitric oxide donors in cardiac arrest and cardiopulmonary resuscitation treatment.
Myocardial Stunning
Suppression of Superoxide-Hydrogen Peroxide Production at Site IQ of Mitochondrial Complex I Attenuates Myocardial Stunning and Improves Postcardiac Arrest Outcomes.
Myocarditis
[Antihypoxic effect of vitamin E and its derivative in rats under modeling of hypoxic conditions of different origin]
Myoclonic Epilepsies, Progressive
Progressive myoclonic epilepsy due to rare mitochondrial ND6 mutation, m.14487T>C.
Myopathies, Nemaline
A novel de novo ACTA1 variant in a patient with nemaline myopathy and mitochondrial Complex I deficiency.
Myopathies, Nemaline
In vitro analysis of rod composition and actin dynamics in inherited myopathies.
Myositis
Cloning of the human mitochondrial 51 kDa subunit (NDUFV1) reveals a 100% antisense homology of its 3'UTR with the 5'UTR of the gamma-interferon inducible protein (IP-30) precursor: is this a link between mitochondrial myopathy and inflammation?
Myositis
Major Histocompatibility Complex I and II Expression and Lymphocytic Subtypes in Muscle of Horses with Immune-Mediated Myositis.
Myxoma
Carney complex 1 with PRKAR1A mutations manifesting as multiple repeated skin myxomas: A case report.
Myxoma
Serp2, an inhibitor of the interleukin-1beta-converting enzyme, is critical in the pathobiology of myxoma virus.
nadh:ubiquinone reductase (h+-translocating) deficiency
"ROS-generating mitochondrial DNA mutations can regulate tumor cell metastasis"-a critical commentary.
nadh:ubiquinone reductase (h+-translocating) deficiency
189th ENMC international workshop complex I deficiency: diagnosis and treatment 20-22 April 2012, Naarden, The Netherlands.
nadh:ubiquinone reductase (h+-translocating) deficiency
31P NMR examination of two patients with NADH-CoQ reductase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
A 31P magnetic resonance spectroscopy study of mitochondrial function in skeletal muscle of patients with Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
A case of ATR-X syndrome with mitochondrial respiratory chain dysfunction.
nadh:ubiquinone reductase (h+-translocating) deficiency
A case of mitochondrial cytopathy with exertion induced dystonia.
nadh:ubiquinone reductase (h+-translocating) deficiency
A case of mitochondrial myopathy, lactic acidosis and complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
A case of Ohtahara syndrome with mitochondrial respiratory chain complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
A catalytic defect in mitochondrial respiratory chain complex I due to a mutation in NDUFS2 in a patient with Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
A Chinese girl with Leigh syndrome: effect of botulinum toxin on dystonia.
nadh:ubiquinone reductase (h+-translocating) deficiency
A common pattern of brain MRI imaging in mitochondrial diseases with complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
A constant and similar assembly defect of mitochondrial respiratory chain complex I allows rapid identification of NDUFS4 mutations in patients with Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
A Drosophila Mitochondrial Complex I Deficiency Phenotype Array.
nadh:ubiquinone reductase (h+-translocating) deficiency
A Drosophila model of mitochondrial disease caused by a complex I mutation that uncouples proton pumping from electron transfer.
nadh:ubiquinone reductase (h+-translocating) deficiency
A False-Positive Diagnosis for the Common MELAS (A3243G) Mutation Caused by a Novel Variant (A3426G) in the ND1 Gene of Mitochondria DNA.
nadh:ubiquinone reductase (h+-translocating) deficiency
A Heterozygous NDUFV1 Variant Aggravates Mitochondrial Complex I Deficiency in a Family with a Homoplasmic ND1 Variant.
nadh:ubiquinone reductase (h+-translocating) deficiency
A high rate (20%-30%) of parental consanguinity in cytochrome-oxidase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
A homozygous mutation in the NDUFS1 gene presents with a mild cavitating leukoencephalopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
A homozygous splicing mutation in ELAC2 suggests phenotypic variability including intellectual disability with minimal cardiac involvement.
nadh:ubiquinone reductase (h+-translocating) deficiency
A homozygous variant in NDUFA8 is associated with developmental delay, microcephaly, and epilepsy due to mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
A LON-ClpP Proteolytic Axis Degrades Complex I to Extinguish ROS Production in Depolarized Mitochondria.
nadh:ubiquinone reductase (h+-translocating) deficiency
A MELAS-associated ND1 mutation causing leber hereditary optic neuropathy and spastic dystonia.
nadh:ubiquinone reductase (h+-translocating) deficiency
A meta-analysis and systematic review of Leigh syndrome: clinical manifestations, respiratory chain enzyme complex deficiency, and gene mutations.
nadh:ubiquinone reductase (h+-translocating) deficiency
A missense mutation in the mitochondrial ND5 gene associated with a Leigh-MELAS overlap syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
A mitochondrial encephalomyopathy with specific deficiencies of two respiratory chain polypeptides and a circulating autoantibody to a mitochondrial matrix protein.
nadh:ubiquinone reductase (h+-translocating) deficiency
A mitochondrial proteomics view of complex I deficiency in Candida albicans.
nadh:ubiquinone reductase (h+-translocating) deficiency
A new mitochondrial DNA mutation in ND3 gene causing severe Leigh syndrome with early lethality.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel de novo ACTA1 variant in a patient with nemaline myopathy and mitochondrial Complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel de novo STXBP1 mutation is associated with mitochondrial complex I deficiency and late-onset juvenile-onset parkinsonism.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel mitochondrial m.14430A>G (MT-ND6, p.W82R) variant causes complex I deficiency and mitochondrial Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel mitochondrial MTND5 frameshift mutation causing isolated complex I deficiency, renal failure and myopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel mutation in NDUFB11 unveils a new clinical phenotype associated with lactic acidosis and sideroblastic anemia.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel mutation in the human complex I NDUFS7 subunit associated with Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel mutation in the mitochondrial tRNA(Pro) gene associated with late-onset ataxia, retinitis pigmentosa, deafness, leukoencephalopathy and complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel mutation in the mitochondrial tRNA(Ser(AGY)) gene associated with mitochondrial myopathy, encephalopathy, and complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel mutation in the thiamine responsive megaloblastic anaemia gene SLC19A2 in a patient with deficiency of respiratory chain complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel mutation of the NDUFS7 gene leads to activation of a cryptic exon and impaired assembly of mitochondrial complex I in a patient with Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel ND3 mitochondrial DNA mutation in three Korean children with basal ganglia lesions and complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel NDUFA1 mutation leads to a progressive mitochondrial complex I-specific neurodegenerative disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel NDUFV1 gene mutation in complex I deficiency in consanguineous siblings with brainstem lesions and Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
A novel recurrent mitochondrial DNA mutation in ND3 gene is associated with isolated complex I deficiency causing Leigh syndrome and dystonia.
nadh:ubiquinone reductase (h+-translocating) deficiency
A Patient with Complex I Deficiency Caused by a Novel ACAD9 Mutation Not Responding to Riboflavin Treatment.
nadh:ubiquinone reductase (h+-translocating) deficiency
A plasma metabolomic signature of Leber hereditary optic neuropathy showing taurine and nicotinamide deficiencies.
nadh:ubiquinone reductase (h+-translocating) deficiency
A rapid method for the isolation of metabolically active mitochondria from rat neurons and astrocytes in primary culture.
nadh:ubiquinone reductase (h+-translocating) deficiency
A recurrent mitochondrial p.Trp22Arg NDUFB3 variant causes a distinctive facial appearance, short stature and a mild biochemical and clinical phenotype.
nadh:ubiquinone reductase (h+-translocating) deficiency
A study of 133 Chinese children with mitochondrial respiratory chain complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Abnormal RNA processing associated with a novel tRNA mutation in mitochondrial DNA. A potential disease mechanism.
nadh:ubiquinone reductase (h+-translocating) deficiency
Acadian variant of Fanconi syndrome is caused by mitochondrial respiratory chain complex I deficiency due to a non-coding mutation in complex I assembly factor NDUFAF6.
nadh:ubiquinone reductase (h+-translocating) deficiency
Acyl-CoA dehydrogenase 9 is required for the biogenesis of oxidative phosphorylation complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
Age-associated mitochondrial complex I deficiency is linked to increased stem cell proliferation rates in the mouse colon.
nadh:ubiquinone reductase (h+-translocating) deficiency
Aggregated ?-synuclein and complex I deficiency: exploration of their relationship in differentiated neurons.
nadh:ubiquinone reductase (h+-translocating) deficiency
AIF, reactive oxygen species, and neurodegeneration: A "complex" problem.
nadh:ubiquinone reductase (h+-translocating) deficiency
alpha-Tocopherol/lipid ratio in blood is decreased in patients with Leber's hereditary optic neuropathy and asymptomatic carriers of the 11778 mtDNA mutation.
nadh:ubiquinone reductase (h+-translocating) deficiency
Altered dopamine metabolism and increased vulnerability to MPTP in mice with partial deficiency of mitochondrial complex I in dopamine neurons.
nadh:ubiquinone reductase (h+-translocating) deficiency
Altered mitochondrial structure and motion dynamics in living cells with energy metabolism defects revealed by real time microscope imaging.
nadh:ubiquinone reductase (h+-translocating) deficiency
Alternative oxidase rescues mitochondria-mediated dopaminergic cell loss in Drosophila.
nadh:ubiquinone reductase (h+-translocating) deficiency
An atypical presentation of ACAD9 deficiency: Diagnosis by whole exome sequencing broadens the phenotypic spectrum and alters treatment approach.
nadh:ubiquinone reductase (h+-translocating) deficiency
An autopsy case of mitochondrial encephalomyopathy with prominent degeneration in olivo-ponto-cerebellar system.
nadh:ubiquinone reductase (h+-translocating) deficiency
An unusual patient with the neonatal Marfan phenotype and mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
An X-chromosome linked mouse model (Ndufa1(S55A)) for systemic partial Complex I deficiency for studying predisposition to neurodegeneration and other diseases.
nadh:ubiquinone reductase (h+-translocating) deficiency
Analysis of functional consequences of haplogroup J polymorphisms m.4216T>C and m.3866T>C in human MT-ND1: Mutagenesis of homologous positions in Escherichia coli.
nadh:ubiquinone reductase (h+-translocating) deficiency
Analysis of mitochondrial DNA sequences in patients with isolated or combined oxidative phosphorylation system deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Analysis of reactive oxygen species and antioxidant defenses in complex I deficient patients revealed a specific increase in superoxide dismutase activity.
nadh:ubiquinone reductase (h+-translocating) deficiency
Analysis of the mitochondrial complex I-v enzyme activities of peripheral leukocytes in oxidative phosphorylation disorders.
nadh:ubiquinone reductase (h+-translocating) deficiency
Analysis of the mitochondrial encoded subunits of complex I in 20 patients with a complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Anatomic and disease specificity of NADH CoQ1 reductase (complex I) deficiency in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Anesthetic Hypersensitivity in a Case-Controlled Series of Patients With Mitochondrial Disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Another "Complex" Case: Complex I Deficiency Secondary to Acyl-CoA Dehydrogenase 9 Mutation.
nadh:ubiquinone reductase (h+-translocating) deficiency
Aplasia of the retinal vessels combined with optic nerve hypoplasia, neonatal epileptic seizures, and lactic acidosis due to mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Apomorphine rescues reactive oxygen species-induced apoptosis of fibroblasts with mitochondrial disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Application of inhibitor titrations for the detection of oxidative phosphorylation defects in saponin-skinned muscle fibers of patients with mitochondrial diseases.
nadh:ubiquinone reductase (h+-translocating) deficiency
Assembly defects induce oxidative stress in inherited mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Assembly factors as a new class of disease genes for mitochondrial complex I deficiency: cause, pathology and treatment options.
nadh:ubiquinone reductase (h+-translocating) deficiency
Assembly of mitochondrial complex I and defects in disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
AtOMA1 Affects the OXPHOS System and Plant Growth in Contrast to Other Newly Identified ATP-Independent Proteases in Arabidopsis Mitochondria.
nadh:ubiquinone reductase (h+-translocating) deficiency
Atypical form of Menkes kinky hair disease with mitochondrial NADH-CoQ reductase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Baculovirus complementation restores a novel NDUFAF2 mutation causing complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Benign mitochondrial encephalomyopathy in a patient with complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Benign mitochondrial myopathy with exercise intolerance in a large multigeneration family due to a homoplasmic m.3250T>C mutation in MTTL1.
nadh:ubiquinone reductase (h+-translocating) deficiency
Bi-allelic Mutations in NDUFA6 Establish Its Role in Early-Onset Isolated Mitochondrial Complex I Deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Bi-allelic pathogenic variants in NDUFC2 cause early-onset Leigh syndrome and stalled biogenesis of complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
Biallelic mutations in NDUFA8 cause complex I deficiency in two siblings with favorable clinical evolution.
nadh:ubiquinone reductase (h+-translocating) deficiency
Biallelic Mutations in TMEM126B Cause Severe Complex I Deficiency with a Variable Clinical Phenotype.
nadh:ubiquinone reductase (h+-translocating) deficiency
Biallelic P4HTM variants associated with HIDEA syndrome and mitochondrial respiratory chain complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Bilateral infantile cataractogenesis in a patient with deficiency of complex I, a mitochondrial electron transport chain enzyme.
nadh:ubiquinone reductase (h+-translocating) deficiency
Biochemical analysis of cybrids expressing mitochondrial DNA from Contursi kindred Parkinson's subjects.
nadh:ubiquinone reductase (h+-translocating) deficiency
Biochemical and genetic analysis of 3-methylglutaconic aciduria type IV: a diagnostic strategy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Biochemical and genetic analysis of Leigh syndrome patients in Korea.
nadh:ubiquinone reductase (h+-translocating) deficiency
Biochemical consequences of two clinically relevant ND-gene mutations in Escherichia coli respiratory complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
Bipolar cell reduction precedes retinal ganglion neuron loss in a complex 1 knockout mouse model.
nadh:ubiquinone reductase (h+-translocating) deficiency
Brain, skeletal muscle and platelet homogenate mitochondrial function in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Broad phenotypic variability in patients with complex I deficiency due to mutations in NDUFS1 and NDUFV1.
nadh:ubiquinone reductase (h+-translocating) deficiency
Btn3 is a negative regulator of Btn2-mediated endosomal protein trafficking and prion curing in yeast.
nadh:ubiquinone reductase (h+-translocating) deficiency
Building a complex complex: Assembly of mitochondrial respiratory chain complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
Ca2+-mobilizing agonists increase mitochondrial ATP production to accelerate cytosolic Ca2+ removal: aberrations in human complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Caenorhabditis elegans expressing the Saccharomyces cerevisiae NADH alternative dehydrogenase Ndi1p, as a tool to identify new genes involved in complex I related diseases.
nadh:ubiquinone reductase (h+-translocating) deficiency
Calcium and ATP handling in human NADH:Ubiquinone oxidoreductase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Can a single subunit yeast NADH dehydrogenase (Ndi1) remedy diseases caused by respiratory complex I defects?
nadh:ubiquinone reductase (h+-translocating) deficiency
Cell biological consequences of mitochondrial NADH: ubiquinone oxidoreductase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Cell-permeable protein therapy for complex I dysfunction.
nadh:ubiquinone reductase (h+-translocating) deficiency
Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Cellular and animal models for mitochondrial complex I deficiency: A focus on the NDUFS4 subunit.
nadh:ubiquinone reductase (h+-translocating) deficiency
Cellular mechanisms of complex I-associated pathology.
nadh:ubiquinone reductase (h+-translocating) deficiency
Cellular rescue-assay aids verification of causative DNA-variants in mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Chapter 9 Reliable assay for measuring complex I activity in human blood lymphocytes and skin fibroblasts.
nadh:ubiquinone reductase (h+-translocating) deficiency
Characterization of mitochondrial FOXRED1 in the assembly of respiratory chain complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
Characterization of the human complex I NDUFB7 and 17.2-kDa cDNAs and mutational analysis of 19 genes of the HP fraction in complex I-deficient-patients.
nadh:ubiquinone reductase (h+-translocating) deficiency
Childhood-Onset Progressive Dystonia With Mitochondrial DNA G14459A Mutation: Efficacy of Long-Term Sodium Succinate Treatment.
nadh:ubiquinone reductase (h+-translocating) deficiency
Chlamydomonas reinhardtii as a plant model system to study mitochondrial complex I dysfunction.
nadh:ubiquinone reductase (h+-translocating) deficiency
Chronic progressive external ophthalmoplegia with NADH-CoQ reductase deficiency: report of a case.
nadh:ubiquinone reductase (h+-translocating) deficiency
CIA30 complex I assembly factor: a candidate for human complex I deficiency?
nadh:ubiquinone reductase (h+-translocating) deficiency
Clinical and molecular features of encephalomyopathy due to the A3302G mutation in the mitochondrial tRNA(Leu(UUR)) gene.
nadh:ubiquinone reductase (h+-translocating) deficiency
Clinical and molecular findings in children with complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Clinical and molecular heterogeneity in patients with mitochondrial encephalomyopathies due to complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Clinical features and neuroradiological findings of mitochondrial pathology in six neonates.
nadh:ubiquinone reductase (h+-translocating) deficiency
Clinical presentation of mitochondrial respiratory chain defects in NADH-coenzyme Q reductase and cytochrome oxidase: clues to pathogenesis of Leigh disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Clinical spectrum, morbidity, and mortality in 113 pediatric patients with mitochondrial disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Clinical, biochemical, and genetic analysis of the mitochondrial respiratory chain complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Clonal expansion of mutated mitochondrial DNA is associated with tumor formation and complex I deficiency in the benign renal oncocytoma.
nadh:ubiquinone reductase (h+-translocating) deficiency
Cloning of the human mitochondrial 51 kDa subunit (NDUFV1) reveals a 100% antisense homology of its 3'UTR with the 5'UTR of the gamma-interferon inducible protein (IP-30) precursor: is this a link between mitochondrial myopathy and inflammation?
nadh:ubiquinone reductase (h+-translocating) deficiency
Codon-specific translational defect caused by a wobble modification deficiency in mutant tRNA from a human mitochondrial disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Coenzyme Q10 supplementation provides mild symptomatic benefit in patients with Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Combined OXPHOS complex I and IV defect, due to mutated complex I assembly factor C20ORF7.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complete Mitochondrial Complex I Deficiency Induces an Up-Regulation of Respiratory Fluxes That Is Abolished by Traces of Functional Complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I (reduced nicotinamide-adenine dinucleotide-coenzyme Q reductase) deficiency in two patients with probable Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I and energy thresholds in the brain.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I assembly: a puzzling problem.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiencies in neurological disorders.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency and dopaminergic neuronal cell loss in parkin-deficient zebrafish (Danio rerio).
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency and Leigh syndrome through the eyes of a clinician.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency due to selective loss of ndufs4 in the mouse heart results in severe hypertrophic cardiomyopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency in association with structural abnormalities of the diaphragm and brain.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency in Parkinson's disease frontal cortex.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency in Persian multiple sclerosis patients.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency is associated with 3243G:C mitochondrial DNA in osteosarcoma cell cybrids.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency primes Bax-dependent neuronal apoptosis through mitochondrial oxidative damage.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency related to T10158C mutation ND3 gene: A further definition of the clinical spectrum.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency with diabetes, Fanconi syndrome and mtDNA deletion.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency, due to NDUFAF4 mutations, causes severe mitochondrial dysfunction and is associated to early death and dysmorphia.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I deficiency: clinical features, biochemistry and molecular genetics.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I is rate-limiting for oxygen consumption in the nerve terminal.
nadh:ubiquinone reductase (h+-translocating) deficiency
Complex I, iron, and ferritin in Parkinson's disease substantia nigra.
nadh:ubiquinone reductase (h+-translocating) deficiency
Compound heterozygous missense and deep intronic variants in NDUFAF6 unraveled by exome sequencing and mRNA analysis.
nadh:ubiquinone reductase (h+-translocating) deficiency
Congenital deficiency of a 20-kDa subunit of mitochondrial complex I in fibroblasts.
nadh:ubiquinone reductase (h+-translocating) deficiency
Congenital lactic acidosis, cerebral cysts and pulmonary hypertension in an infant with FOXRED1 related complex 1 deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Congenital lactic acidosis, cerebral cysts and pulmonary hypertension in an infant with FOXRED1 related complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Congenital neurogenic muscular atrophy in megaconial myopathy due to a mutation in CHKB gene.
nadh:ubiquinone reductase (h+-translocating) deficiency
Congenital ocular motor apraxia associated with myopathy, external hydrocephalus and NADH dehydrogenase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Constitutive Reprogramming of Fibroblast Mitochondrial Metabolism in Pulmonary Hypertension.
nadh:ubiquinone reductase (h+-translocating) deficiency
Correction to: The antioxidant Trolox restores mitochondrial membrane potential and Ca2+-stimulated ATP production in human complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Corrigendum to "Mitigation of NADH:ubiquinone oxidoreductase deficiency by chronic Trolox treatment" [Biochimica et Biophysica Acta 1777/7-8 (2008) 853-859].
nadh:ubiquinone reductase (h+-translocating) deficiency
Corrigendum: Hepatic S6K1 Partially Regulates Lifespan of Mice with Mitochondrial Complex I Deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Cotargeting of Mitochondrial Complex I and Bcl-2 Shows Antileukemic Activity against Acute Myeloid Leukemia Cells Reliant on Oxidative Phosphorylation.
nadh:ubiquinone reductase (h+-translocating) deficiency
Cyclic adenosine monophosphate-dependent phosphorylation of mammalian mitochondrial proteins: enzyme and substrate characterization and functional role.
nadh:ubiquinone reductase (h+-translocating) deficiency
Cyclosporin inhibition of apoptosis induced by mitochondrial complex I toxins.
nadh:ubiquinone reductase (h+-translocating) deficiency
Cystic Leucoencephalopathy in NDUFV1 Mutation.
nadh:ubiquinone reductase (h+-translocating) deficiency
D-galactose induces a mitochondrial complex I deficiency in mouse skeletal muscle: potential benefits of nutrient combination in ameliorating muscle impairment.
nadh:ubiquinone reductase (h+-translocating) deficiency
De novo mutations in the mitochondrial ND3 gene as a cause of infantile mitochondrial encephalopathy and complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Decreased agonist-stimulated mitochondrial ATP production caused by a pathological reduction in endoplasmic reticulum calcium content in human complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Decylubiquinol impedes mitochondrial respiratory chain complex I activity.
nadh:ubiquinone reductase (h+-translocating) deficiency
Deep intronic TIMMDC1 variant delays diagnosis of rapidly progressive complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Deep Sequencing Reveals Novel Genetic Variants in Children with Acute Liver Failure and Tissue Evidence of Impaired Energy Metabolism.
nadh:ubiquinone reductase (h+-translocating) deficiency
Defective NDUFA9 as a novel cause of neonatally fatal complex I disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Defects in mitochondrial ATP synthesis in dystrophin-deficient mdx skeletal muscles may be caused by complex I insufficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Deficiency in Mitochondrial Complex I Activity Due to Ndufs6 Gene Trap Insertion Induces Renal Disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Deficiency of respiratory chain complex I in Hashimoto thyroiditis.
nadh:ubiquinone reductase (h+-translocating) deficiency
Degeneration of human oncogenes and mitochondrial genes occurs in cells that exhibit age-related pathology.
nadh:ubiquinone reductase (h+-translocating) deficiency
Degradation of mitochondrial structure and deficiency of complex I were associated with the transgenic CMS of rice.
nadh:ubiquinone reductase (h+-translocating) deficiency
Deletion of the Complex I Subunit NDUFS4 Adversely Modulates Cellular Differentiation.
nadh:ubiquinone reductase (h+-translocating) deficiency
Dementia in Parkinson's disease is associated with enhanced mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Demonstration of a new pathogenic mutation in human complex I deficiency: a 5-bp duplication in the nuclear gene encoding the 18-kD (AQDQ) subunit.
nadh:ubiquinone reductase (h+-translocating) deficiency
Determination of enzyme activities for prenatal diagnosis of respiratory chain deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Diagnosis of complex I deficiency in patients with lactic acidemia using skin fibroblast cultures.
nadh:ubiquinone reductase (h+-translocating) deficiency
Diagnosis of mitochondrial disorders by concomitant next-generation sequencing of the exome and mitochondrial genome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Diverse phenotype in patients with complex I deficiency due to mutations in NDUFB11.
nadh:ubiquinone reductase (h+-translocating) deficiency
DJ-1 deficiency in astrocytes selectively enhances mitochondrial Complex I inhibitor-induced neurotoxicity.
nadh:ubiquinone reductase (h+-translocating) deficiency
DJ-1 knock-down impairs astrocyte mitochondrial function.
nadh:ubiquinone reductase (h+-translocating) deficiency
Dopamine transporter SPECT in patients with mitochondrial disorders.
nadh:ubiquinone reductase (h+-translocating) deficiency
Down-Regulation of NDUFB9 Promotes Breast Cancer Cell Proliferation, Metastasis by Mediating Mitochondrial Metabolism.
nadh:ubiquinone reductase (h+-translocating) deficiency
Downregulation of apoptosis-inducing factor in Harlequin mice induces progressive and severe optic atrophy which is durably prevented by AAV2-AIF1 gene therapy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Dynamics of Human Mitochondrial Complex I Assembly: Implications for Neurodegenerative Diseases.
nadh:ubiquinone reductase (h+-translocating) deficiency
Early-onset cataracts, spastic paraparesis, and ataxia caused by a novel mitochondrial tRNAGlu (MT-TE) gene mutation causing severe complex I deficiency: a clinical, molecular, and neuropathologic study.
nadh:ubiquinone reductase (h+-translocating) deficiency
Early-onset ophthalmoplegia in Leigh-like syndrome due to NDUFV1 mutations.
nadh:ubiquinone reductase (h+-translocating) deficiency
Echinacoside selectively rescues complex I inhibition-induced mitochondrial respiratory impairment via enhancing complex II activity.
nadh:ubiquinone reductase (h+-translocating) deficiency
Effect of various agents on adenosine triphosphate synthesis in mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
ELAC2 Mutations Cause a Mitochondrial RNA Processing Defect Associated with Hypertrophic Cardiomyopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Epilepsia partialis continua associated with NADH-coenzyme Q reductase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Evaluation of enzymatic assays and compounds affecting ATP production in mitochondrial respiratory chain complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Evaluation of mitochondrial bioenergetics, dynamics, endoplasmic reticulum-mitochondria crosstalk, and reactive oxygen species in fibroblasts from patients with complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Evidence for mitochondrial dysfunction in Parkinson's disease--a critical appraisal.
nadh:ubiquinone reductase (h+-translocating) deficiency
Evidence of a wide spectrum of cardiac involvement due to ACAD9 mutations: Report on nine patients.
nadh:ubiquinone reductase (h+-translocating) deficiency
Evoked potentials in children with oxidative metabolic defects leading to Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Evolution of FOXRED1, an FAD-dependent oxidoreductase necessary for NADH:ubiquinone oxidoreductase (Complex I) assembly.
nadh:ubiquinone reductase (h+-translocating) deficiency
Evolution, structure and membrane association of NDUFAF6, an assembly factor for NADH:ubiquinone oxidoreductase (Complex I).
nadh:ubiquinone reductase (h+-translocating) deficiency
Excessive formation of hydroxyl radicals and aldehydic lipid peroxidation products in cultured skin fibroblasts from patients with complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Exercise intolerance due to a nonsense mutation in the mtDNA ND4 gene.
nadh:ubiquinone reductase (h+-translocating) deficiency
Exome sequencing expedites disease gene discovery.
nadh:ubiquinone reductase (h+-translocating) deficiency
Exome sequencing identifies ACAD9 mutations as a cause of complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Exome sequencing identifies complex I NDUFV2 mutations as a novel cause of Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Exome sequencing of patients with histiocytoid cardiomyopathy reveals a de novo NDUFB11 mutation that plays a role in the pathogenesis of histiocytoid cardiomyopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Expression of GRIM-19 in unexplained recurrent spontaneous abortion and possible pathogenesis.
nadh:ubiquinone reductase (h+-translocating) deficiency
Expression of yeast NDI1 rescues a Drosophila complex I assembly defect.
nadh:ubiquinone reductase (h+-translocating) deficiency
External alternative NADH:ubiquinone oxidoreductase redirected to the internal face of the mitochondrial inner membrane rescues complex I deficiency in Yarrowia lipolytica.
nadh:ubiquinone reductase (h+-translocating) deficiency
Facial Nerve Recovery in KbDb and C1q Knockout Mice: A Role for Histocompatibility Complex 1.
nadh:ubiquinone reductase (h+-translocating) deficiency
Familial mitochondrial complex I deficiency with an abnormal mitochondrial encoded protein.
nadh:ubiquinone reductase (h+-translocating) deficiency
Fatal hypertensive crisis as presentation of mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Fatal mitochondrial myopathy, lactic acidosis, and complex I deficiency associated with a heteroplasmic A --> G mutation at position 3251 in the mitochondrial tRNALeu(UUR) gne.
nadh:ubiquinone reductase (h+-translocating) deficiency
Fermentation and alternative respiration compensate for NADH dehydrogenase deficiency in a prokaryotic model of DJ-1-associated Parkinsonism.
nadh:ubiquinone reductase (h+-translocating) deficiency
Findings in muscle in complex I (NADH coenzyme Q reductase) deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
FOXRED1, encoding an FAD-dependent oxidoreductase complex-I-specific molecular chaperone, is mutated in infantile-onset mitochondrial encephalopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Free radical scavengers protect dopaminergic cell lines from apoptosis induced by complex I inhibitors.
nadh:ubiquinone reductase (h+-translocating) deficiency
Free radicals and mitochondrial dysfunction in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Free radicals-mediated damage in transmitochondrial cells harboring the T14487C mutation in the ND6 gene of mtDNA.
nadh:ubiquinone reductase (h+-translocating) deficiency
Fulminant neurological deterioration in a neonate with Leigh syndrome due to a maternally transmitted missense mutation in the mitochondrial ND3 gene.
nadh:ubiquinone reductase (h+-translocating) deficiency
Functional expression of the single subunit NADH dehydrogenase in mitochondria in vivo: a potential therapy for complex I deficiencies.
nadh:ubiquinone reductase (h+-translocating) deficiency
Gait analysis in a mouse model resembling Leigh disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Gene Expression Profiling of Mitochondrial Oxidative Phosphorylation (OXPHOS) Complex I in Friedreich Ataxia (FRDA) Patients.
nadh:ubiquinone reductase (h+-translocating) deficiency
Genetic and biochemical findings in Chinese children with Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Genetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystonia.
nadh:ubiquinone reductase (h+-translocating) deficiency
Genetic counselling and prenatal diagnosis in disorders of the mitochondrial energy metabolism.
nadh:ubiquinone reductase (h+-translocating) deficiency
Genetic diversity of NDUFV1-dependent mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Genome sequencing and RNA-seq analyses of mitochondrial complex I deficiency revealed Alu insertion-mediated deletion in NDUFV2.
nadh:ubiquinone reductase (h+-translocating) deficiency
Genotype in the 24-kDa subunit gene (NDUFV2) of mitochondrial complex I and susceptibility to Parkinson disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Genotyping microsatellite DNA markers at putative disease loci in inbred/multiplex families with respiratory chain complex I deficiency allows rapid identification of a novel nonsense mutation (IVS1nt -1) in the NDUFS4 gene in Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Glia are critical for the neuropathology of complex I deficiency in Drosophila.
nadh:ubiquinone reductase (h+-translocating) deficiency
Glial lipid droplets and neurodegeneration in a Drosophila model of complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Glucose Transporter Type I Deficiency Causing Mitochondrial Dysfunction.
nadh:ubiquinone reductase (h+-translocating) deficiency
GRIM-19 deficiency promotes clear cell renal cell carcinoma progression and is associated with high TNM stage and Fuhrman grade.
nadh:ubiquinone reductase (h+-translocating) deficiency
Heart specific knockout of Ndufs4 ameliorates ischemia reperfusion injury.
nadh:ubiquinone reductase (h+-translocating) deficiency
Hepatic S6K1 Partially Regulates Lifespan of Mice with Mitochondrial Complex I Deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Heterozygous mutation in the X chromosomal NDUFA1 gene in a girl with complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
High incidence and variable clinical outcome of cardiac hypertrophy due to ACAD9 mutations in childhood.
nadh:ubiquinone reductase (h+-translocating) deficiency
High resolution respirometry of permeabilized skeletal muscle fibers in the diagnosis of neuromuscular disorders.
nadh:ubiquinone reductase (h+-translocating) deficiency
High-throughput, pooled sequencing identifies mutations in NUBPL and FOXRED1 in human complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Homoplasmy of a mitochondrial 3697G>A mutation causes Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Human complex I defects can be resolved by monoclonal antibody analysis into distinct subunit assembly patterns.
nadh:ubiquinone reductase (h+-translocating) deficiency
Human complex I defects in neurodegenerative diseases.
nadh:ubiquinone reductase (h+-translocating) deficiency
Human complex I deficiency: clinical spectrum and involvement of oxygen free radicals in the pathogenicity of the defect.
nadh:ubiquinone reductase (h+-translocating) deficiency
Human mitochondrial complex I assembles through the combination of evolutionary conserved modules: a framework to interpret complex I deficiencies.
nadh:ubiquinone reductase (h+-translocating) deficiency
Human mitochondrial complex I deficiency: investigating transcriptional responses by microarray.
nadh:ubiquinone reductase (h+-translocating) deficiency
Human NADH:ubiquinone oxidoreductase deficiency: radical changes in mitochondrial morphology?
nadh:ubiquinone reductase (h+-translocating) deficiency
Human NADH:ubiquinone oxidoreductase.
nadh:ubiquinone reductase (h+-translocating) deficiency
Human xenomitochondrial cybrids. Cellular models of mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Identification and characterization of a common set of complex I assembly intermediates in mitochondria from patients with complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Identification and Characterization of New Variants in FOXRED1 Gene Expands the Clinical Spectrum Associated with Mitochondrial Complex I Deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Identification of mitochondria translation elongation factor Tu as a contributor to oxidative damage of postburn myocardium.
nadh:ubiquinone reductase (h+-translocating) deficiency
Impaired mitochondrial beta-oxidation in a patient with an abnormality of the respiratory chain. Studies in skeletal muscle mitochondria.
nadh:ubiquinone reductase (h+-translocating) deficiency
Impairment of the mitochondrial respiratory chain activity in diethylnitrosamine-induced rat hepatomas: possible involvement of oxygen free radicals.
nadh:ubiquinone reductase (h+-translocating) deficiency
Improvement of lesions shown on MRI and CT scan by administration of dichloroacetate in patients with Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
In vitro analysis of rod composition and actin dynamics in inherited myopathies.
nadh:ubiquinone reductase (h+-translocating) deficiency
Increase in proteins involved in mitochondrial fission, mitophagy, proteolysis and antioxidant response in type I endometrial cancer as an adaptive response to respiratory complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Increased mitochondrial ATP production capacity in brain of healthy mice and a mouse model of isolated complex I deficiency after isoflurane anesthesia.
nadh:ubiquinone reductase (h+-translocating) deficiency
Increased Protein S-Glutathionylation in Leber's Hereditary Optic Neuropathy (LHON).
nadh:ubiquinone reductase (h+-translocating) deficiency
Increased risk for cardiorespiratory failure associated with the A3302G mutation in the mitochondrial DNA encoded tRNALeu(UUR) gene.
nadh:ubiquinone reductase (h+-translocating) deficiency
Increasing fat in the diet does not improve muscle performance in patients with mitochondrial myopathy due to complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Inherited complex I deficiency is associated with faster protein diffusion in the matrix of moving mitochondria.
nadh:ubiquinone reductase (h+-translocating) deficiency
Inhibiting cytosolic translation and autophagy improves health in mitochondrial disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Inhibition of mitochondrial function induces an integrated stress response in oligodendroglia.
nadh:ubiquinone reductase (h+-translocating) deficiency
Inhibition of mitochondrial Na+-Ca2+ exchange restores agonist-induced ATP production and Ca2+ handling in human complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Inhibition of neuronal mitochondrial complex I or lysosomal glucocerebrosidase is associated with increased dopamine and serotonin turnover.
nadh:ubiquinone reductase (h+-translocating) deficiency
Initiation of neuronal damage by complex I deficiency and oxidative stress in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Insights into the pathogenic character of a common NUBPL branch-site mutation associated with mitochondrial disease and complex I deficiency using a yeast model.
nadh:ubiquinone reductase (h+-translocating) deficiency
Intractable secretory diarrhea in a Japanese boy with mitochondrial respiratory chain complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Intragenic inversion of mtDNA: a new type of pathogenic mutation in a patient with mitochondrial myopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Investigating complex I deficiency in Purkinje cells and synapses in patients with mitochondrial disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Is there a special relationship between complex I activity and nigral neuronal loss in Parkinson's disease? A critical reappraisal.
nadh:ubiquinone reductase (h+-translocating) deficiency
Isoflurane anesthetic hypersensitivity and progressive respiratory depression in a mouse model with isolated mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Isolated and combined deficiencies of NADH dehydrogenase (complex I) in muscle tissue of children with mitochondrial myopathies.
nadh:ubiquinone reductase (h+-translocating) deficiency
Isolated complex I deficiency in children: clinical, biochemical and genetic aspects.
nadh:ubiquinone reductase (h+-translocating) deficiency
Isolated deficiencies of OXPHOS complexes I and IV are identified accurately and quickly by simple enzyme activity immunocapture assays.
nadh:ubiquinone reductase (h+-translocating) deficiency
Isolated mitochondrial complex I deficiency: explorative data analysis of patient cell parameters.
nadh:ubiquinone reductase (h+-translocating) deficiency
L-dihydroxyphenylalanine and complex I deficiency in Parkinson's disease brain.
nadh:ubiquinone reductase (h+-translocating) deficiency
Lack of complex I activity in human cells carrying a mutation in MtDNA-encoded ND4 subunit is corrected by the Saccharomyces cerevisiae NADH-quinone oxidoreductase (NDI1) gene.
nadh:ubiquinone reductase (h+-translocating) deficiency
Lactate disposal via gluconeogenesis is increased during exercise in patients with mitochondrial myopathy due to complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Large-scale deletion and point mutations of the nuclear NDUFV1 and NDUFS1 genes in mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Late-Onset Leigh Syndrome due to NDUFV1 Mutation in a 10-Year-Old Boy Initially Presenting with Ataxia.
nadh:ubiquinone reductase (h+-translocating) deficiency
Late-onset Leigh syndrome in a patient with mitochondrial complex I NDUFS8 mutations.
nadh:ubiquinone reductase (h+-translocating) deficiency
Leber hereditary optic neuropathy due to a new ND1 mutation.
nadh:ubiquinone reductase (h+-translocating) deficiency
Leber hereditary optic neuropathy: involvement of the mitochondrial ND1 gene and evidence for an intragenic suppressor mutation.
nadh:ubiquinone reductase (h+-translocating) deficiency
Leber's hereditary optic neuropathy and complex I deficiency in muscle.
nadh:ubiquinone reductase (h+-translocating) deficiency
Leigh disease associated with a novel mitochondrial DNA ND5 mutation.
nadh:ubiquinone reductase (h+-translocating) deficiency
Leigh disease caused by the mitochondrial DNA G14459A mutation in unrelated families.
nadh:ubiquinone reductase (h+-translocating) deficiency
Leigh disease with brainstem involvement in complex I deficiency due to assembly factor NDUFAF2 defect.
nadh:ubiquinone reductase (h+-translocating) deficiency
Leigh syndrome associated with mitochondrial complex I deficiency due to a novel mutation in the NDUFS1 gene.
nadh:ubiquinone reductase (h+-translocating) deficiency
Leigh syndrome associated with mitochondrial complex I deficiency due to novel mutations In NDUFV1 and NDUFS2.
nadh:ubiquinone reductase (h+-translocating) deficiency
Leigh Syndrome in Childhood: Neurologic Progression and Functional Outcome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Leigh syndrome with cytochrome-c oxidase deficiency and a single T insertion nt 5537 in the mitochondrial tRNATrp gene.
nadh:ubiquinone reductase (h+-translocating) deficiency
Leigh syndrome: clinical features and biochemical and DNA abnormalities.
nadh:ubiquinone reductase (h+-translocating) deficiency
Lethal infantile mitochondrial disease with isolated complex I deficiency in fibroblasts but with combined complex I and IV deficiencies in muscle.
nadh:ubiquinone reductase (h+-translocating) deficiency
Leukodystrophy associated with mitochondrial complex I deficiency due to a novel mutation in the NDUFAF1 gene.
nadh:ubiquinone reductase (h+-translocating) deficiency
LHON/MELAS overlap syndrome associated with a mitochondrial MTND1 gene mutation.
nadh:ubiquinone reductase (h+-translocating) deficiency
Lifetime exercise intolerance with lactic acidosis as key manifestation of novel compound heterozygous ACAD9 mutations causing complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Liver Pathology in Mitochondrial Complex I Deficiency from Bi-Allelic Mutations in NDUFS2: A Report of Findings at Autopsy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Liver-specific mitochondrial respiratory chain complex I deficiency in fatal influenza encephalopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia.
nadh:ubiquinone reductase (h+-translocating) deficiency
Loss of Mitochondrial Ndufs4 in Striatal Medium Spiny Neurons Mediates Progressive Motor Impairment in a Mouse Model of Leigh Syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Loss-of-Function NUBPL Mutation May Link Parkinson's Disease to Recessive Complex I Deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Low mutant load of mitochondrial DNA G13513A mutation can cause Leigh's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mammalian Mitochondrial Complex I Structure and Disease-Causing Mutations.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mechanism of cell death caused by complex I defects in a rat dopaminergic cell line.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mechanism of neurodegeneration of neurons with mitochondrial DNA mutations.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mesencephalic complex I deficiency does not correlate with parkinsonism in mitochondrial DNA maintenance disorders.
nadh:ubiquinone reductase (h+-translocating) deficiency
Metabolic flexibility of mitochondrial respiratory chain disorders predicted by computer modelling.
nadh:ubiquinone reductase (h+-translocating) deficiency
Metabolic interventions against complex I deficiency in MELAS syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Metabolome and proteome profiling of complex I deficiency induced by rotenone.
nadh:ubiquinone reductase (h+-translocating) deficiency
Metallothionein 1 Overexpression Does Not Protect Against Mitochondrial Disease Pathology in Ndufs4 Knockout Mice.
nadh:ubiquinone reductase (h+-translocating) deficiency
Metallothionein isoform 2A expression is inducible and protects against ROS-mediated cell death in rotenone-treated HeLa cells.
nadh:ubiquinone reductase (h+-translocating) deficiency
Metformin delays neurological symptom onset in a mouse model of neuronal complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mice with mitochondrial complex I deficiency develop a fatal encephalomyopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
MidA is a putative methyltransferase that is required for mitochondrial complex I function.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitigation of NADH: ubiquinone oxidoreductase deficiency by chronic Trolox treatment.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondria deficient in complex I activity are depolarized by hydrogen peroxide in nerve terminals: relevance to Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondria in the etiology and pathogenesis of Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondria in the nervous system: From Health to Disease, Part I.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial and cytosolic thiol redox state are not detectably altered in isolated human NADH:ubiquinone oxidoreductase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial and nuclear genes as the cause of complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial Ca2+ homeostasis in human NADH:ubiquinone oxidoreductase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I and II activities of lymphocytes and platelets in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I defects increase ubiquitin in substantia nigra.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial Complex I Deficiency among Egyptian Pediatric Patients with Steroid-Resistant Nephrotic Syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency and ATP/ADP ratio in lymphocytes of amyotrophic lateral sclerosis patients.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency and cardiovascular diseases: current evidence and future directions.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency caused by a deleterious NDUFA11 mutation.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency enhances skeletal myogenesis but impairs insulin signaling through SIRT1 inactivation.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency in a female with multiplex arthrogryposis congenita.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency in GDAP1-related autosomal dominant Charcot-Marie-Tooth disease (CMT2K).
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial Complex I Deficiency in Schizophrenia and Bipolar Disorder and Medication Influence.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency in the epileptic focus of patients with temporal lobe epilepsy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency increases protein acetylation and accelerates heart failure.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency leads to inflammation and retinal ganglion cell death in the Ndufs4 mouse.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency leads to the retardation of early embryonic development in Ndufs4 knockout mice.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency of nuclear origin I. Structural genes.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency of nuclear origin II. Non-structural genes.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I dysfunction in rat heart with aging: critical role of reactive oxygen species and cardiolipin.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial complex I-linked disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial disease and epilepsy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial disease associated with complex I (NADH-CoQ oxidoreductase) deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial disorders in children: toward development of small-molecule treatment strategies.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial DNA and primary mitochondrial dysfunction in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial DNA in focal dystonia: a cybrid analysis.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial DNA mutations in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS).
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial DNA polymorphisms and haplogroups in Parkinson's disease and control individuals with a similar genetic background.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial DNA transmission of the mitochondrial defect in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial dynamics in human NADH:ubiquinone oxidoreductase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial dysfunction in neurodegeneration.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial dysfunction in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial dysfunction triggers the pathogenesis of Parkinson's disease in neuronal C/EBP? transgenic mice.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial Dysfunctions in Type I Endometrial Carcinoma: Exploring Their Role in Oncogenesis and Tumor Progression.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial encephalomyopathies in childhood. II. Clinical manifestations and syndromes.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial Encephalomyopathy Due to a Novel Mutation in ACAD9.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial encephalomyopathy. Association with an NADH dehydrogenase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial fluidity matters. Focus on "Inherited complex I deficiency is associated with faster protein diffusion in the matrix of moving mitochondria".
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial function in Parkinson's disease. The Royal Kings and Queens Parkinson's Disease Research Group.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial function, GSH and iron in neurodegeneration and Lewy body diseases.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial GRIM-19 deficiency facilitates gastric cancer metastasis through oncogenic ROS-NRF2-HO-1 axis via a NRF2-HO-1 loop.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial involvement in Parkinson's disease, Huntington's disease, hereditary spastic paraplegia and Friedreich's ataxia.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial iron-sulfur cluster biogenesis from molecular understanding to clinical disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial myopathies: morphological and biochemical studies in human muscle cultures.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial myopathy with tRNA(Leu(UUR)) mutation and complex I deficiency responsive to riboflavin.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes syndrome and NADH-CoQ reductase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial NADH-coenzyme Q reductase deficiency in Leigh's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial network complexity and pathological decrease in complex I activity are tightly correlated in isolated human complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial Neurogastrointestinal Encephalopathy: Clinical, Biochemical and Molecular Study in Three Egyptian Patients.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial oxidative phosphorylation disorders presenting in neonates: clinical manifestations and enzymatic and molecular diagnoses.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial respiratory chain complex I deficiency causes intractable gastrointestinal symptoms.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial respiratory chain complex I deficiency with clinical and biochemical features of long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial respiratory chain enzyme assay and DNA analysis in peripheral blood leukocytes for the etiological study of Chinese children with Leigh syndrome due to complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial respiratory chain function in multiple system atrophy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial respiratory complex I deficiency simulating spinal muscular atrophy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial respiratory failure in skeletal muscle from patients with Parkinson's disease and multiple system atrophy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mitochondrial tRNA(Leu(UUR)) mutation m.3302A > G presenting as childhood-onset severe myopathy: threshold determination through segregation study.
nadh:ubiquinone reductase (h+-translocating) deficiency
Modelling the effects of age-related mtDNA mutation accumulation; complex I deficiency, superoxide and cell death.
nadh:ubiquinone reductase (h+-translocating) deficiency
Molecular base of biochemical complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Molecular characterization and mutational analysis of the human B17 subunit of the mitochondrial respiratory chain complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
Molecular diagnosis in mitochondrial complex I deficiency using exome sequencing.
nadh:ubiquinone reductase (h+-translocating) deficiency
Molecular Gene Therapy: Overexpression of the Alternative NADH Dehydrogenase NDI1 Restores Overall Physiology in a Fungal Model of Respiratory Complex I Deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Molecular remedy of complex I defects: rotenone-insensitive internal NADH-quinone oxidoreductase of Saccharomyces cerevisiae mitochondria restores the NADH oxidase activity of complex I-deficient mammalian cells.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mouse models for nuclear DNA-encoded mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mouse models of mitochondrial complex I dysfunction.
nadh:ubiquinone reductase (h+-translocating) deficiency
MR spectroscopy and serial magnetic resonance imaging in a patient with mitochondrial cystic leukoencephalopathy due to complex I deficiency and NDUFV1 mutations and mild clinical course.
nadh:ubiquinone reductase (h+-translocating) deficiency
MtDNA and nuclear mutations affecting oxidative phosphorylation: correlating severity of clinical defect with extent of bioenergetic compromise.
nadh:ubiquinone reductase (h+-translocating) deficiency
MtDNA mutations are a common cause of severe disease phenotypes in children with Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
MTO1-Deficient Mouse Model Mirrors the Human Phenotype Showing Complex I Defect and Cardiomyopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Multi-OMICS study of a CHCHD10 variant causing ALS demonstrates metabolic rewiring and activation of endoplasmic reticulum and mitochondrial unfolded protein responses.
nadh:ubiquinone reductase (h+-translocating) deficiency
Multiomic analysis elucidates Complex I deficiency caused by a deep intronic variant in NDUFB10.
nadh:ubiquinone reductase (h+-translocating) deficiency
Multisystem mitochondrial diseases due to mutations in mtDNA-encoded subunits of complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
Muscle respiratory chain enzyme activities in parkinson's disease and in multisystem extrapyramidal disorders with parkinsonism as the main clinical feature.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutant NDUFS3 subunit of mitochondrial complex I causes Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutant NDUFV2 subunit of mitochondrial complex I causes early onset hypertrophic cardiomyopathy and encephalopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutated NDUFS6 is the cause of fatal neonatal lactic acidemia in Caucasus Jews.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutation of c20orf7 disrupts complex I assembly and causes lethal neonatal mitochondrial disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutation screening of 75 candidate genes in 152 complex I deficiency cases identifies pathogenic variants in 16 genes including NDUFB9.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutations in Complex I Assembly Factor TMEM126B Result in Muscle Weakness and Isolated Complex I Deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutations in human nuclear genes encoding for subunits of mitochondrial respiratory complex I: the NDUFS4 gene.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex I assembly protein, cause fatal neonatal mitochondrial disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutations in the accessory subunit NDUFB10 result in isolated complex I deficiency and illustrate the critical role of intermembrane space import for complex I holoenzyme assembly.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutations in the complex I NDUFS2 gene of patients with cardiomyopathy and encephalomyopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutations in the gene encoding C8orf38 block complex I assembly by inhibiting production of the mitochondria-encoded subunit ND1.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutations in the ND5 subunit of complex I of the mitochondrial DNA are a frequent cause of oxidative phosphorylation disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Mutations in the NDUFS4 gene of mitochondrial complex I alter stability of the splice variants.
nadh:ubiquinone reductase (h+-translocating) deficiency
Myelopathy in Two Brothers with Respiratory Chain Disorder-Severe Complex 1 Deficiency with Atlantoaxial Dislocation and Long Spinal Arachnoid Cyst: A New Unreported Association.
nadh:ubiquinone reductase (h+-translocating) deficiency
NADH dehydrogenase deficiency in an apoptosis-resistant mutant isolated from a human HL-60 leukemia cell line.
nadh:ubiquinone reductase (h+-translocating) deficiency
NADH dehydrogenase deficiency results in low respiration rate and improved aerobic growth of Zymomonas mobilis.
nadh:ubiquinone reductase (h+-translocating) deficiency
NADH-coenzyme Q reductase (complex I) deficiency: heterogeneity in phenotype and biochemical findings.
nadh:ubiquinone reductase (h+-translocating) deficiency
Natural disease course and genotype-phenotype correlations in Complex I deficiency caused by nuclear gene defects: what we learned from 130 cases.
nadh:ubiquinone reductase (h+-translocating) deficiency
Natural Product Screening Reveals Naphthoquinone Complex I Bypass Factors.
nadh:ubiquinone reductase (h+-translocating) deficiency
NDUFA10 mutations cause complex I deficiency in a patient with Leigh disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
NDUFA2 complex I mutation leads to Leigh disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
NDUFA9 point mutations cause a variable mitochondrial complex I assembly defect.
nadh:ubiquinone reductase (h+-translocating) deficiency
NDUFAF4 variants are associated with Leigh syndrome and cause a specific mitochondrial complex I assembly defect.
nadh:ubiquinone reductase (h+-translocating) deficiency
NDUFB8 Mutations Cause Mitochondrial Complex I Deficiency in Individuals with Leigh-like Encephalomyopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
NDUFS4 mutations cause Leigh syndrome with predominant brainstem involvement.
nadh:ubiquinone reductase (h+-translocating) deficiency
NDUFS6 mutations are a novel cause of lethal neonatal mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
NDUFS6 related Leigh syndrome: a case report and review of the literature.
nadh:ubiquinone reductase (h+-translocating) deficiency
NDUFS8-related Complex I Deficiency Extends Phenotype from "PEO Plus" to Leigh Syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Necrotizing encephalopathy and macrocephaly with mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Nemaline rods and complex I deficiency in three infants with hypotonia, motor delay and failure to thrive.
nadh:ubiquinone reductase (h+-translocating) deficiency
Neonatal multiorgan failure due to ACAD9 mutation and complex I deficiency with mitochondrial hyperplasia in liver, cardiac myocytes, skeletal muscle, and renal tubules.
nadh:ubiquinone reductase (h+-translocating) deficiency
Neonatal onset of mitochondrial disorders in 129 patients: clinical and laboratory characteristics and a new approach to diagnosis.
nadh:ubiquinone reductase (h+-translocating) deficiency
Neurofibromatosis Type 1: A Novel NF1 Mutation Associated with Mitochondrial Complex I Deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Neuronal complex I deficiency occurs throughout the Parkinson's disease brain, but is not associated with neurodegeneration or mitochondrial DNA damage.
nadh:ubiquinone reductase (h+-translocating) deficiency
Neurospora strains harboring mitochondrial disease-associated mutations in iron-sulfur subunits of complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
New familial mitochondrial encephalopathy with macrocephaly, cardiomyopathy, and complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
New insights into the cause of Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
New pattern of brain MRI lesions in isolated complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Next-generation sequencing in molecular diagnosis: NUBPL mutations highlight the challenges of variant detection and interpretation.
nadh:ubiquinone reductase (h+-translocating) deficiency
Nicotinamide adenine dinucleotides permeate through mitochondrial membranes in human Epstein-Barr virus-transformed lymphocytes.
nadh:ubiquinone reductase (h+-translocating) deficiency
No evidence of a role for mitochondrial complex I in Helicobacter pylori pathogenesis.
nadh:ubiquinone reductase (h+-translocating) deficiency
Nonsense and missense mutation of mitochondrial ND6 gene promotes cell migration and invasion in human lung adenocarcinoma.
nadh:ubiquinone reductase (h+-translocating) deficiency
Nonviability of cells with oxidative defects in galactose medium: a screening test for affected patient fibroblasts.
nadh:ubiquinone reductase (h+-translocating) deficiency
Novel insights into the role of Neurospora crassa NDUFAF2, an evolutionarily conserved mitochondrial complex I assembly factor.
nadh:ubiquinone reductase (h+-translocating) deficiency
Novel mitochondrial DNA ND5 mutation in a patient with clinical features of MELAS and MERRF.
nadh:ubiquinone reductase (h+-translocating) deficiency
Novel MT-ND Gene Variants Causing Adult-Onset Mitochondrial Disease and Isolated Complex I Deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Novel MTND1 mutations cause isolated exercise intolerance, complex I deficiency and increased assembly factor expression.
nadh:ubiquinone reductase (h+-translocating) deficiency
Novel mutations in the mitochondrial complex I assembly gene NDUFAF5 reveal heterogeneous phenotypes.
nadh:ubiquinone reductase (h+-translocating) deficiency
Novel mutations in the NDUFS1 gene cause low residual activities in human complex I deficiencies.
nadh:ubiquinone reductase (h+-translocating) deficiency
Novel mutations of ND genes in complex I deficiency associated with mitochondrial encephalopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Novel Nrf2-Inducer Prevents Mitochondrial Defects and Oxidative Stress in Friedreich's Ataxia Models.
nadh:ubiquinone reductase (h+-translocating) deficiency
NUBPL mitochondrial disease: new patients and review of the genetic and clinical spectrum.
nadh:ubiquinone reductase (h+-translocating) deficiency
NUBPL mutations in patients with complex I deficiency and a distinct MRI pattern.
nadh:ubiquinone reductase (h+-translocating) deficiency
Nuclear DNA origin of mitochondrial complex I deficiency in fatal infantile lactic acidosis evidenced by transnuclear complementation of cultured fibroblasts.
nadh:ubiquinone reductase (h+-translocating) deficiency
Nuclear genes of human complex I of the mitochondrial electron transport chain: state of the art.
nadh:ubiquinone reductase (h+-translocating) deficiency
Ohtahara syndrome and respiratory chain complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Ophthalmologic presentation of oxidative phosphorylation diseases of childhood.
nadh:ubiquinone reductase (h+-translocating) deficiency
Optic atrophy 1 mediates mitochondria remodeling and dopaminergic neurodegeneration linked to complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Optic atrophy-associated TMEM126A is an assembly factor for the ND4-module of mitochondrial complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
Optic neuropathy, cardiomyopathy, cognitive disability in patients with a homozygous mutation in the nuclear MTO1 and a mitochondrial MT-TF variant.
nadh:ubiquinone reductase (h+-translocating) deficiency
Oxidative stress and Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Oxidative stress contributes differentially to the pathophysiology of Charcot-Marie-Tooth disease type 2K.
nadh:ubiquinone reductase (h+-translocating) deficiency
Oxidative stress in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Parkinson's disease mutations in PINK1 result in decreased Complex I activity and deficient synaptic function.
nadh:ubiquinone reductase (h+-translocating) deficiency
Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage.
nadh:ubiquinone reductase (h+-translocating) deficiency
Partial loss of complex I due to NDUFS4 deficiency augments myocardial reperfusion damage by increasing mitochondrial superoxide/hydrogen peroxide production.
nadh:ubiquinone reductase (h+-translocating) deficiency
Partial NADH dehydrogenase defect presenting as spastic cerebral palsy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Pathogenic Bi-allelic Mutations in NDUFAF8 Cause Leigh Syndrome with an Isolated Complex I Deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Pathogenic mutations in NUBPL affect complex I activity and cold tolerance in the yeast model Yarrowia lipolytica.
nadh:ubiquinone reductase (h+-translocating) deficiency
Pharmacological targeting of mitochondrial complex I deficiency: The cellular level and beyond.
nadh:ubiquinone reductase (h+-translocating) deficiency
Phenotype-genotype correlations in Leigh syndrome: new insights from a multicentre study of 96 patients.
nadh:ubiquinone reductase (h+-translocating) deficiency
Photoreceptors in a mouse model of Leigh syndrome are capable of normal light-evoked signaling.
nadh:ubiquinone reductase (h+-translocating) deficiency
Physical Exercise and Mitochondrial Disease: Insights From a Mouse Model.
nadh:ubiquinone reductase (h+-translocating) deficiency
PINK1 function in health and disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Platelet mitochondrial function in Parkinson's disease. The Royal Kings and Queens Parkinson Disease Research Group.
nadh:ubiquinone reductase (h+-translocating) deficiency
Polyunsaturated fatty acid-enriched diet therapy for a child with epilepsy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Predominant cerebellar volume loss as a neuroradiologic feature of pediatric respiratory chain defects.
nadh:ubiquinone reductase (h+-translocating) deficiency
Prenatal diagnosis of NADH:ubiquinone oxidoreductase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Progressive cavitating leukoencephalopathy associated with respiratory chain complex I deficiency and a novel mutation in NDUFS1.
nadh:ubiquinone reductase (h+-translocating) deficiency
Progressive encephalopathy and complex I deficiency associated with mutations in MTND1.
nadh:ubiquinone reductase (h+-translocating) deficiency
Progressive increase of the mutated mitochondrial DNA fraction in Kearns-Sayre syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Progressive optic atrophy in a retinal ganglion cell-specific mouse model of complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Prostaglandin A1 inhibits rotenone-induced apoptosis in SH-SY5Y cells.
nadh:ubiquinone reductase (h+-translocating) deficiency
Pure myopathy with enlarged mitochondria associated to a new mutation in MTND2 gene.
nadh:ubiquinone reductase (h+-translocating) deficiency
Quantifying small molecule phenotypic effects using mitochondrial morpho-functional fingerprinting and machine learning.
nadh:ubiquinone reductase (h+-translocating) deficiency
Quantitation of a mitochondrial DNA deletion in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Quantitative analysis of proteins of metabolism by reverse phase protein microarrays identifies potential biomarkers of rare neuromuscular diseases.
nadh:ubiquinone reductase (h+-translocating) deficiency
Ragged-red fibers and complex I deficiency in a neonate with arthrogryposis congenita.
nadh:ubiquinone reductase (h+-translocating) deficiency
Randomized, double-blind, placebo-controlled pilot trial of reduced coenzyme Q10 for Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Rapamycin enhances survival in a Drosophila model of mitochondrial disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Rapid screening for nuclear genes mutations in isolated respiratory chain complex I defects.
nadh:ubiquinone reductase (h+-translocating) deficiency
Recessive Mutations in NDUFA2 Cause Mitochondrial Leukoencephalopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Recessive spastic paraparesis associated with complex I deficiency due to MTHFR mutations.
nadh:ubiquinone reductase (h+-translocating) deficiency
Redefining the roles of mitochondrial DNA-encoded subunits in respiratory Complex I assembly.
nadh:ubiquinone reductase (h+-translocating) deficiency
Replacement of the C6ORF66 Assembly Factor (NDUFAF4) Restores Complex I Activity in Patient Cells.
nadh:ubiquinone reductase (h+-translocating) deficiency
Repopulation of rho0 cells with mitochondria from a patient with a mitochondrial DNA point mutation in tRNA(Gly) results in respiratory chain dysfunction.
nadh:ubiquinone reductase (h+-translocating) deficiency
Respiratory chain and mitochondrial deoxyribonucleic acid in blood cells from patients with focal and generalized dystonia.
nadh:ubiquinone reductase (h+-translocating) deficiency
Respiratory chain complex I deficiency caused by mitochondrial DNA mutations.
nadh:ubiquinone reductase (h+-translocating) deficiency
Respiratory chain complex I deficiency due to NDUFA12 mutations as a new cause of Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Respiratory chain complex I deficiency in an infant with infantile spasms.
nadh:ubiquinone reductase (h+-translocating) deficiency
Respiratory chain complex I deficiency in an infant with Ohtahara syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Respiratory chain complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Respiratory chain complex I deficiency: an underdiagnosed energy generation disorder.
nadh:ubiquinone reductase (h+-translocating) deficiency
Respiratory chain complex I is a mitochondrial tumor suppressor of oncocytic tumors.
nadh:ubiquinone reductase (h+-translocating) deficiency
Respiratory chain deficiency in a female with Aicardi-Goutières syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Respiratory complex I deficiency induces drought tolerance by impacting leaf stomatal and hydraulic conductances.
nadh:ubiquinone reductase (h+-translocating) deficiency
Respiratory complex I deficiency results in low nitric oxide levels, induction of hemoglobin and upregulation of fermentation pathways.
nadh:ubiquinone reductase (h+-translocating) deficiency
Resting oxygen consumption and in vivo ADP are increased in myopathy due to complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Restoration of mitochondrial function in cells with complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Resveratrol attenuates oxidative stress in mitochondrial Complex I deficiency: Involvement of SIRT3.
nadh:ubiquinone reductase (h+-translocating) deficiency
Riboflavin enhances the assembly of mitochondrial cytochrome c oxidase in C. elegans NADH-ubiquinone oxidoreductase mutants.
nadh:ubiquinone reductase (h+-translocating) deficiency
Riboflavin-responsive complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Riboflavin-responsive oxidative phosphorylation complex I deficiency caused by defective ACAD9: new function for an old gene.
nadh:ubiquinone reductase (h+-translocating) deficiency
Rotenone destroys dopaminergic neurons and induces parkinsonian symptoms in rats.
nadh:ubiquinone reductase (h+-translocating) deficiency
Secondary inhibition of multiple NAD-requiring dehydrogenases in respiratory chain complex I deficiency: possible metabolic markers for the primary defect.
nadh:ubiquinone reductase (h+-translocating) deficiency
Secondary metabolic effects in complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Selection and Characterization of Palmitic Acid Responsive Patients with an OXPHOS Complex I Defect.
nadh:ubiquinone reductase (h+-translocating) deficiency
Sengers syndrome: six novel AGK mutations in seven new families and review of the phenotypic and mutational spectrum of 29 patients.
nadh:ubiquinone reductase (h+-translocating) deficiency
Sequence analysis of nuclear genes encoding functionally important complex I subunits in children with encephalomyopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Sequence variations in the NDUFA1 gene encoding a subunit of complex I of the respiratory chain.
nadh:ubiquinone reductase (h+-translocating) deficiency
Serine Catabolism Feeds NADH when Respiration Is Impaired.
nadh:ubiquinone reductase (h+-translocating) deficiency
Severe complex I deficiency in a case of neonatal-onset lactic acidosis and fatal liver failure.
nadh:ubiquinone reductase (h+-translocating) deficiency
Severe infantile leigh syndrome associated with a rare mitochondrial ND6 mutation, m.14487T>C.
nadh:ubiquinone reductase (h+-translocating) deficiency
Severe nigrostriatal degeneration without clinical parkinsonism in patients with polymerase gamma mutations.
nadh:ubiquinone reductase (h+-translocating) deficiency
SLC25A10 biallelic mutations in intractable epileptic encephalopathy with complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Smoking and mitochondrial function: a model for environmental toxins.
nadh:ubiquinone reductase (h+-translocating) deficiency
SOD2 gene transfer protects against optic neuropathy induced by deficiency of complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
Sodium salicylate protects against rotenone-induced Parkinsonism in rats.
nadh:ubiquinone reductase (h+-translocating) deficiency
Spare respiratory capacity rather than oxidative stress regulates glutamate excitotoxicity after partial respiratory inhibition of mitochondrial complex I with rotenone.
nadh:ubiquinone reductase (h+-translocating) deficiency
Spectrophotometric assay for complex I of the respiratory chain in tissue samples and cultured fibroblasts.
nadh:ubiquinone reductase (h+-translocating) deficiency
Sporadic heteroplasmic single 5.5 kb mitochondrial DNA deletion associated with cerebellar ataxia, hypogonadotropic hypogonadism, choroidal dystrophy, and mitochondrial respiratory chain complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Studies on the pathogenesis of Parkinson's disease in Japan.
nadh:ubiquinone reductase (h+-translocating) deficiency
Subcomplex Ilambda specifically controls integrated mitochondrial functions in Caenorhabditis elegans.
nadh:ubiquinone reductase (h+-translocating) deficiency
Subcutaneous microdialysis for children - safe biochemical tissue monitoring based on a minimal traumatizing no touch insertion technique.
nadh:ubiquinone reductase (h+-translocating) deficiency
Successful Medical Therapy for Hypophosphatemic Rickets due to Mitochondrial Complex I Deficiency Induced de Toni-Debré-Fanconi Syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Successful pregnancy in a patient with mitochondrial cardiomyopathy due to ACAD9 deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Successful treatment of infantile-onset ACAD9-related cardiomyopathy with a combination of sodium pyruvate, beta-blocker, and coenzyme Q10.
nadh:ubiquinone reductase (h+-translocating) deficiency
Successful treatment of pure myopathy, associated with complex I deficiency, with riboflavin and carnitine.
nadh:ubiquinone reductase (h+-translocating) deficiency
Succinic acidemia: a new syndrome of organic acidemia associated with congenital lactic acidosis and decreased NADH-cytochrome c reductase activity.
nadh:ubiquinone reductase (h+-translocating) deficiency
Sugar transport regulation: comparative characterization of the effect of NADH CoQ reductase deficiency in two cell culture systems.
nadh:ubiquinone reductase (h+-translocating) deficiency
Superoxide production is inversely related to complex I activity in inherited complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Systemic infantile complex I deficiency with fatal outcome in two brothers.
nadh:ubiquinone reductase (h+-translocating) deficiency
Targeting NAD+ Metabolism as Interventions for Mitochondrial Disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
TAT-Conjugated NDUFS8 Can Be Transduced into Mitochondria in a Membrane-Potential-Independent Manner and Rescue Complex I Deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Teaching NeuroImages: rapidly progressive leukoencephalopathy in mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Teaching NeuroImages: Spinal cord gray matter involvement in complex I deficiency mitochondriopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
The 8993 mtDNA mutation: heteroplasmy and clinical presentation in three families.
nadh:ubiquinone reductase (h+-translocating) deficiency
The accumulation of assembly intermediates of the mitochondrial complex I matrix arm is reduced by limiting glucose uptake in a neuronal-like model of MELAS syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
The addition of ketone bodies alleviates mitochondrial dysfunction by restoring complex I assembly in a MELAS cellular model.
nadh:ubiquinone reductase (h+-translocating) deficiency
The analysis of antioxidant expression during muscle atrophy induced by hindlimb suspension in mice.
nadh:ubiquinone reductase (h+-translocating) deficiency
The antioxidant Trolox restores mitochondrial membrane potential and Ca2+ -stimulated ATP production in human complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
The assembly pathway of complex I in Arabidopsis thaliana.
nadh:ubiquinone reductase (h+-translocating) deficiency
The clinical spectrum of the m.10191T>C mutation in complex I-deficient Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
The effects of idebenone on mitochondrial bioenergetics.
nadh:ubiquinone reductase (h+-translocating) deficiency
The energy-transducing NADH: quinone oxidoreductase, complex I.
nadh:ubiquinone reductase (h+-translocating) deficiency
The expanding clinical spectrum of mitochondrial diseases.
nadh:ubiquinone reductase (h+-translocating) deficiency
The first nuclear-encoded complex I mutation in a patient with Leigh syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
The G13513A mutation in the ND5 gene of mitochondrial DNA as a common cause of MELAS or Leigh syndrome: evidence from 12 cases.
nadh:ubiquinone reductase (h+-translocating) deficiency
The human nuclear-encoded acyl carrier subunit (NDUFAB1) of the mitochondrial complex I in human pathology.
nadh:ubiquinone reductase (h+-translocating) deficiency
The impact of reactive oxygen species and genetic mitochondrial mutations in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
The importance of liver biopsy in the investigation of possible mitochondrial respiratory chain disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
The m.3890G>A/MT-ND1 mtDNA rare pathogenic variant: Expanding clinical and MRI phenotypes.
nadh:ubiquinone reductase (h+-translocating) deficiency
The mitochondrial 13513G > A mutation is most frequent in Leigh syndrome combined with reduced complex I activity, optic atrophy and/or Wolff-Parkinson-White.
nadh:ubiquinone reductase (h+-translocating) deficiency
The mitochondrial 13513G>A mutation is associated with Leigh disease phenotypes independent of complex I deficiency in muscle.
nadh:ubiquinone reductase (h+-translocating) deficiency
The mitochondrial DNA G13513A MELAS mutation in the NADH dehydrogenase 5 gene is a frequent cause of Leigh-like syndrome with isolated complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
The molecular basis of human complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
The nuclear-encoded human NADH:ubiquinone oxidoreductase NDUFA8 subunit: cDNA cloning, chromosomal localization, tissue distribution, and mutation detection in complex-I-deficient patients.
nadh:ubiquinone reductase (h+-translocating) deficiency
The nuoM arg368his mutation in NADH:ubiquinone oxidoreductase from Rhodobacter capsulatus: a model for the human nd4-11778 mtDNA mutation associated with Leber's hereditary optic neuropathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families.
nadh:ubiquinone reductase (h+-translocating) deficiency
The respiratory complexes I from the mitochondria of two Pichia species.
nadh:ubiquinone reductase (h+-translocating) deficiency
The role of mitochondria in the regulation of hypoxia-inducible factor 1 expression during hypoxia.
nadh:ubiquinone reductase (h+-translocating) deficiency
The unique neuroradiology of complex I deficiency due to NDUFA12L defect.
nadh:ubiquinone reductase (h+-translocating) deficiency
The use of targeted genomic capture and massively parallel sequencing in diagnosis of Chinese Leukoencephalopathies.
nadh:ubiquinone reductase (h+-translocating) deficiency
The variability of the harlequin mouse phenotype resembles that of human mitochondrial-complex I-deficiency syndromes.
nadh:ubiquinone reductase (h+-translocating) deficiency
Therapeutic applications of the TAT-mediated protein transduction system for complex I deficiency and other mitochondrial diseases.
nadh:ubiquinone reductase (h+-translocating) deficiency
Therapy of complex I deficiency: peripheral neuropathy during dichloroacetate therapy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Tissue specific defect of complex I of the mitochondrial respiratory chain.
nadh:ubiquinone reductase (h+-translocating) deficiency
Toxic effects of dopamine metabolism in Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Transcriptional changes in OXPHOS complex I deficiency are related to anti-oxidant pathways and could explain the disturbed calcium homeostasis.
nadh:ubiquinone reductase (h+-translocating) deficiency
Treatment of complex I deficiency with riboflavin.
nadh:ubiquinone reductase (h+-translocating) deficiency
Treatment of experimental NADH ubiquinone reductase deficiency with menadione.
nadh:ubiquinone reductase (h+-translocating) deficiency
Triacylglycerol infusion does not improve hyperlactemia in resting patients with mitochondrial myopathy due to complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Triacylglycerol infusion improves exercise endurance in patients with mitochondrial myopathy due to complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Two cases of NADH-coenzyme Q reductase deficiency: relationship to MELAS syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Two molecular pathways initiate mitochondria-dependent dopaminergic neurodegeneration in experimental Parkinson's disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Ultrastructural examination of skin biopsies may assist in diagnosing mitochondrial cytopathy when muscle biopsies yield negative results.
nadh:ubiquinone reductase (h+-translocating) deficiency
Uncoupling protein-4 (UCP4) increases ATP supply by interacting with mitochondrial Complex II in neuroblastoma cells.
nadh:ubiquinone reductase (h+-translocating) deficiency
Understanding mitochondrial complex I assembly in health and disease.
nadh:ubiquinone reductase (h+-translocating) deficiency
Unregulated mitochondrial GSK3beta activity results in NADH: ubiquinone oxidoreductase deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Update on clinical aspects and treatment of selected vitamin-responsive disorders II (riboflavin and CoQ(10)).
nadh:ubiquinone reductase (h+-translocating) deficiency
Use of the Ketogenic Diet to Treat Intractable Epilepsy in Mitochondrial Disorders.
nadh:ubiquinone reductase (h+-translocating) deficiency
Use of the NADH-quinone oxidoreductase (NDI1) gene of Saccharomyces cerevisiae as a possible cure for complex I defects in human cells.
nadh:ubiquinone reductase (h+-translocating) deficiency
Using a quantitative quadruple immunofluorescent assay to diagnose isolated mitochondrial Complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Vascular involvement in mitochondrial myopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Visual function, ocular motility and ocular characteristics in patients with mitochondrial complex I deficiency.
nadh:ubiquinone reductase (h+-translocating) deficiency
Vitamin-responsive complex I deficiency in a myopathic patient with increased activity of the terminal respiratory chain and lactic acidosis.
nadh:ubiquinone reductase (h+-translocating) deficiency
Warburg-like effect is a hallmark of complex I assembly defects.
nadh:ubiquinone reductase (h+-translocating) deficiency
Whole Exome Sequencing Identifies the Genetic Basis of Late-Onset Leigh Syndrome in a Patient with MRI but Little Biochemical Evidence of a Mitochondrial Disorder.
nadh:ubiquinone reductase (h+-translocating) deficiency
Whole genome and exome sequencing identify NDUFV2 mutations as a new cause of progressive cavitating leukoencephalopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
Whole-exome Sequencing Helps the Diagnosis and Treatment in Children with Neurodevelopmental Delay Accompanied Unexplained Dyspnea.
nadh:ubiquinone reductase (h+-translocating) deficiency
Whole-Exome Sequencing Identifies a Variant of the Mitochondrial MT-ND1 Gene Associated with epileptic encephalopathy: West syndrome evolving to Lennox-Gastaut Syndrome.
nadh:ubiquinone reductase (h+-translocating) deficiency
Widening the Heterogeneity of Leigh Syndrome: Clinical, Biochemical, and Neuroradiologic Features in a Patient Harboring a NDUFA10 Mutation.
nadh:ubiquinone reductase (h+-translocating) deficiency
X-linked NDUFA1 gene mutations associated with mitochondrial encephalomyopathy.
nadh:ubiquinone reductase (h+-translocating) deficiency
[A case of complex I deficiency with episodic respiratory distress]
nadh:ubiquinone reductase (h+-translocating) deficiency
[A case of Leigh syndrome associated with respiratory chain complex I deficiency due to mitochondrial gene 13513G>A mutation]
nadh:ubiquinone reductase (h+-translocating) deficiency
[Complex I deficiency due to mutations in nuclear-encoded subunit genes]
nadh:ubiquinone reductase (h+-translocating) deficiency
[Complex I deficiency due to NDUFS8 gene mutation]
nadh:ubiquinone reductase (h+-translocating) deficiency
[Disappearance of ALS from Guam: implications for exogenous causes]
nadh:ubiquinone reductase (h+-translocating) deficiency
[Electron transfer complex I deficiency]
nadh:ubiquinone reductase (h+-translocating) deficiency
[Ergometric and pathologic study of a family with complex I deficiency]
nadh:ubiquinone reductase (h+-translocating) deficiency
[High resolution respirometry in diagnostic of mitochondrial disorders caused by mitochondrial complex I deficiency].
nadh:ubiquinone reductase (h+-translocating) deficiency
[Intrahepatic cholestasis due to mitochondrial respiratory chain complex I deficiency in a Chinese boy].
nadh:ubiquinone reductase (h+-translocating) deficiency
[Mitochondrial encephalomyopathy of late presentation with progressive ophthalmoplegia, tremor and diffuse leukoencephalopathy]
nadh:ubiquinone reductase (h+-translocating) deficiency
[Mitochondrial encephalopathy due to complex I deficiency. Brain tissue biopsy findings and clinical course following pharmacological]
nadh:ubiquinone reductase (h+-translocating) deficiency
[Mitochondrial function and mitochondrial DNA in a series of 64 patients suspected of having mitochondrial myopathy]
nadh:ubiquinone reductase (h+-translocating) deficiency
[Mitochondrial respiratory chain complex I deficiency due to 10191T>C mutation in ND3 gene].
nadh:ubiquinone reductase (h+-translocating) deficiency
[Relationship between heroin spongiform leucoencephalopathy and respiratory chain complex I deficiency].
Neoplasm Metastasis
A Positive Step toward Understanding Double-Negative Metastatic Prostate Cancer.
Neoplasm Metastasis
Allosteric and ATP-Competitive Inhibitors of mTOR Effectively Suppress Tumor Progression-Associated Epithelial-Mesenchymal Transition in the Kidneys of Tsc2+/- Mice.
Neoplasm Metastasis
Association of predicted pathogenic mutations in mitochondrial ND genes with distant metastasis in NSCLC and colon cancer.
Neoplasm Metastasis
Down-regulation of a Mitochondrial Micropeptide MPM Promotes Hepatoma Metastasis by Enhancing Mitochondrial Complex I Activity.
Neoplasm Metastasis
Down-regulation of GRIM-19 is associated with STAT3 overexpression in breast carcinomas.
Neoplasm Metastasis
Down-Regulation of NDUFB9 Promotes Breast Cancer Cell Proliferation, Metastasis by Mediating Mitochondrial Metabolism.
Neoplasm Metastasis
Effects of plasmid-based Stat3-specific short hairpin RNA and GRIM-19 on PC-3M tumor cell growth.
Neoplasm Metastasis
Expression of total and phospho 4EBP1 in metastatic and non-metastatic renal cell carcinoma.
Neoplasm Metastasis
GRIM-19 deficiency promotes clear cell renal cell carcinoma progression and is associated with high TNM stage and Fuhrman grade.
Neoplasm Metastasis
GRIM-19 inhibition induced autophagy through activation of ERK and HIF-1? not STAT3 in Hela cells.
Neoplasm Metastasis
GRIM-19 inhibits v-Src-induced cell motility by interfering with cytoskeletal restructuring.
Neoplasm Metastasis
GRIM-19 over-expression represses the proliferation and invasion of orthotopically implanted hepatocarcinoma tumors associated with downregulation of Stat3 signaling.
Neoplasm Metastasis
GRIM-19 Restores Cervical Cancer Cell Senescence by Repressing hTERT Transcription.
Neoplasm Metastasis
GRIM-19: A master regulator of cytokine induced tumor suppression, metastasis and energy metabolism.
Neoplasm Metastasis
IMPAD1 functions as mitochondrial electron transport inhibitor that prevents ROS production and promotes lung cancer metastasis through the AMPK-Notch1-HEY1 pathway.
Neoplasm Metastasis
Integration of mTOR and estrogen-ERK2 signaling in lymphangioleiomyomatosis pathogenesis.
Neoplasm Metastasis
Lymphangioleiomyomatosis Biomarkers Linked to Lung Metastatic Potential and Cell Stemness.
Neoplasm Metastasis
MCT4 is induced by metastasis-enhancing pathogenic mitochondrial NADH dehydrogenase gene mutations and can be a therapeutic target.
Neoplasm Metastasis
Mitochondrial complex I activity and NAD+/NADH balance regulate breast cancer progression.
Neoplasm Metastasis
Mitochondrial GRIM-19 as a potential therapeutic target for STAT3-dependent carcinogenesis of gastric cancer.
Neoplasm Metastasis
Mitochondrial GRIM-19 deficiency facilitates gastric cancer metastasis through oncogenic ROS-NRF2-HO-1 axis via a NRF2-HO-1 loop.
Neoplasm Metastasis
mTORC2 Suppresses GSK3-Dependent Snail Degradation to Positively Regulate Cancer Cell Invasion and Metastasis.
Neoplasm Metastasis
Nicotinamide phosphoribosyltransferase can affect metastatic activity and cell adhesive functions by regulating integrins in breast cancer.
Neoplasm Metastasis
Nonsense and missense mutation of mitochondrial ND6 gene promotes cell migration and invasion in human lung adenocarcinoma.
Neoplasm Metastasis
Re: The Polycomb Repressor Complex 1 Drives Double-Negative Prostate Cancer Metastasis by Coordinating Stemness and Immune Suppression.
Neoplasm Metastasis
Redox state of adipose tissue for patients with gastric cancer and its connection with the body mass index and distance from the tumor.
Neoplasm Metastasis
S100A4 alters metabolism and promotes invasion of lung cancer cells by up-regulating mitochondrial complex I protein NDUFS2.
Neoplasm Metastasis
The Polycomb Repressor Complex 1 Drives Double-Negative Prostate Cancer Metastasis by Coordinating Stemness and Immune Suppression.
Neoplasm Metastasis
Transglutaminase 2 mediates hypoxia-induced selective mRNA translation via polyamination of 4EBPs.
Neoplasm Metastasis
Tumor Microenvironment Modulates Immunological Outcomes of Myeloid Cells with mTORC1 Disruption.
Neoplasm Metastasis
Tumor suppressive protein gene associated with retinoid-interferon-induced mortality (GRIM)-19 inhibits src-induced oncogenic transformation at multiple levels.
Neoplasm Metastasis
Upregulation of the GRIM-19 gene suppresses invasion and metastasis of human gastric cancer SGC-7901 cell line.
Neoplasm Micrometastasis
Flura-seq identifies organ-specific metabolic adaptations during early metastatic colonization.
Neoplasms
"ROS-generating mitochondrial DNA mutations can regulate tumor cell metastasis"-a critical commentary.
Neoplasms
18F-FAZA PET imaging response tracks the reoxygenation of tumors in mice upon treatment with the mitochondrial complex I inhibitor BAY 87-2243.
Neoplasms
3-Benzyl-5-((2-nitrophenoxy) methyl)-dihydrofuran-2(3H)-one suppresses Fc?RI-mediated mast cell degranulation via the inhibition of mTORC2-Akt signaling.
Neoplasms
4EBP1/eIF4E and p70S6K/RPS6 axes play critical and distinct roles in hepatocarcinogenesis driven by AKT and N-Ras proto-oncogenes in mice.
Neoplasms
?9-THC increases endogenous AHA1 expression in rat cerebellum and may modulate CB1 receptor function during chronic use.
Neoplasms
A cisplatin-based platinum(IV) prodrug containing a glutathione s-transferase inhibitor to reverse cisplatin-resistance in non-small cell lung cancer.
Neoplasms
A dual-targeting, apoptosis-inducing organometallic half-sandwich iridium anticancer complex.
Neoplasms
A genome-wide enrichment screen identifies NUMA1-loss as a resistance mechanism against mitotic cell-death induced by BMI1 inhibition.
Neoplasms
A genome-wide siRNA screen in mammalian cells for regulators of S6 phosphorylation.
Neoplasms
A Humanized Bone Niche Model Reveals Bone Tissue Preservation Upon Targeting Mitochondrial Complex I in Pseudo-Orthotopic Osteosarcoma.
Neoplasms
A mTORC1-mediated cyst(e)ine sensing mechanism governing GPX4 synthesis and ferroptosis.
Neoplasms
A mutation screening of oncogenes, tumor suppressor gene TP53 and nuclear encoded mitochondrial complex I genes in oncocytic thyroid tumors.
Neoplasms
A mutation threshold distinguishes the anti- tumorigenic effects of the mitochondrial gene MTND1, an oncojanus function.
Neoplasms
A new Cu(II)-O-Carvacrotinate complex: Synthesis, characterization and biological activity.
Neoplasms
A novel binuclear hydrazone-based Cd(II) complex is a strong pro-apoptotic inducer with significant activity against 2D and 3D pancreatic cancer stem cells.
Neoplasms
A novel mitochondrial inhibitor blocks MAPK pathway and overcomes MAPK inhibitor-resistance in melanoma.
Neoplasms
A novel one-dimensional manganese(II) coordination polymer containing both dicyanamide and pyrazinamide ligands: Synthesis, spectroscopic investigations, X-ray studies and evaluation of biological activities.
Neoplasms
A novel silver iodide metalo-drug: Experimental and computational modelling assessment of its interaction with intracellular DNA, lipoxygenase and glutathione.
Neoplasms
A novel thiophene-3-carboxamide analog of annonaceous acetogenin exhibits antitumor activity via inhibition of mitochondrial complex I.
Neoplasms
A Pan-Cancer Landscape Analysis Reveals a Subset of Endometrial Stromal and Pediatric Tumors Defined by Internal Tandem Duplications of BCOR.
Neoplasms
A photoactivatable Ru (II) complex bearing 2,9-diphenyl-1,10-phenanthroline: A potent chemotherapeutic drug inducing apoptosis in triple negative human breast adenocarcinoma cells.
Neoplasms
A proteomic analysis reveals the loss of expression of the cell death regulatory gene GRIM-19 in human renal cell carcinomas.
Neoplasms
A review of the basics of mitochondrial bioenergetics, metabolism, and related signaling pathways in cancer cells: Therapeutic targeting of tumor mitochondria with lipophilic cationic compounds.
Neoplasms
A Rhodium(III)-Based Inhibitor of Lysine-Specific Histone Demethylase 1 as an Epigenetic Modulator in Prostate Cancer Cells.
Neoplasms
A Short-Lived but Highly Cytotoxic Vanadium(V) Complex as a Potential Drug Lead for Brain Cancer Treatment by Intratumoral Injections.
Neoplasms
A Small Molecule Inhibitor of Polycomb Repressive Complex 1 Inhibits Ubiquitin Signaling at DNA Double-strand Breaks.
Neoplasms
A system to identify inhibitors of mTOR signaling using high-resolution growth analysis in Saccharomyces cerevisiae.
Neoplasms
Acidosis Drives the Reprogramming of Fatty Acid Metabolism in Cancer Cells through Changes in Mitochondrial and Histone Acetylation.
Neoplasms
Activation of DNA Damage Tolerance Pathways May Improve Immunotherapy of Mesothelioma.
Neoplasms
Active-site mTOR inhibitors augment HSV1-dICP0 infection in cancer cells via dysregulated eIF4E/4E-BP axis.
Neoplasms
AG311, a small molecule inhibitor of complex I and hypoxia-induced HIF-1? stabilization.
Neoplasms
Ago-RIP-Seq identifies Polycomb repressive complex I member CBX7 as a major target of miR-375 in prostate cancer progression.
Neoplasms
Allosteric and ATP-Competitive Inhibitors of mTOR Effectively Suppress Tumor Progression-Associated Epithelial-Mesenchymal Transition in the Kidneys of Tsc2+/- Mice.
Neoplasms
Alterations in expression levels of genes in p53-related pathways determined using RNA-Seq analysis in patients with breast cancer following CIK therapy.
Neoplasms
Alterations of Oxidative Phosphorylation Complexes in Papillary Thyroid Carcinoma.
Neoplasms
Altered expression of a metformin-mediated radiation response in SA-NH and FSa tumor cells treated under in vitro and in vivo growth conditions.
Neoplasms
Ameliorative Effect of Coenzyme Q10 and/or Candesartan on Carboplatin-Induced Nephrotoxicity: Roles of Apoptosis, Transforming Growth Factor-?1, Nuclear Factor Kappa-B And The Nrf2/HO-1 Pathway
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Amino acid-dependent NPRL2 interaction with Raptor determines mTOR Complex 1 activation.
Neoplasms
AMPK-dependent phosphorylation is required for transcriptional activation of TFEB and TFE3.
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An A10398G mitochondrial DNA alteration is related to increased risk of breast cancer, and associates with Her2 positive receptor.
Neoplasms
An Acidity-Unlocked Magnetic Nanoplatform Enables Self-Boosting ROS Generation through Upregulation of Lactate for Imaging-Guided Highly Specific Chemodynamic Therapy.
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An anti-prostate cancer benzofuran-conjugated iridium(III) complex as a dual inhibitor of STAT3 and NF-?B.
Neoplasms
An anticancer agent, pyrvinium pamoate inhibits the NADH-fumarate reductase system--a unique mitochondrial energy metabolism in tumour microenvironments.
Neoplasms
An inherited mitochondrial DNA disruptive mutation shifts to homoplasmy in oncocytic tumor cells.
Neoplasms
An inverted CAV1 (caveolin 1) topology defines novel autophagy-dependent exosome secretion from prostate cancer cells.
Neoplasms
Analysis of cytotoxic activity at short incubation times reveals profound differences among Annonaceus acetogenins, inhibitors of mitochondrial Complex I.
Neoplasms
Analysis of the mitochondrial proteome of cybrid cells harbouring a truncative mitochondrial DNA mutation in respiratory complex I.
Neoplasms
Anti-cancer agent 3-bromopyruvate reduces growth of MPNST and inhibits metabolic pathways in a representative in-vitro model.
Neoplasms
Antibody-mediated neutralization of soluble MIC significantly enhances CTLA4 blockade therapy.
Neoplasms
Anticancer action of cubé insecticide: correlation for rotenoid constituents between inhibition of NADH:ubiquinone oxidoreductase and induced ornithine decarboxylase activities.
Neoplasms
Anticancer activities of the ruthenium carboxylato, amido and pyridine complexes.
Neoplasms
Anticancer Activity Assessment and DNA Binding Properties of Two Binuclear Platinum (II) Complexes using Spectroscopic and Molecular Simulation Approaches.
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Antitumor activity of annonaceous acetogenins in HepS and S180 xenografts bearing mice.
Neoplasms
Antitumor activity of phenylene bridged binuclear bis(imino-quinolyl)palladium(II) and platinum(II) complexes.
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Antitumoral synergism between a copper(II) complex and cisplatin improves in vitro and in vivo anticancer activity against melanoma, lung and breast cancer cells.
Neoplasms
Application of machine learning to large in-vitro databases to identify cancer cell characteristics: telomerase reverse transcriptase (TERT) expression.
Neoplasms
Architecture and dynamics of the autophagic phosphatidylinositol 3-kinase complex.
Neoplasms
Ascofuranone suppresses invasion and F-actin cytoskeleton organization in cancer cells by inhibiting the mTOR complex 1 signaling pathway.
Neoplasms
Association of focal adhesion kinase with tuberous sclerosis complex 2 in the regulation of s6 kinase activation and cell growth.
Neoplasms
Association of predicted pathogenic mutations in mitochondrial ND genes with distant metastasis in NSCLC and colon cancer.
Neoplasms
Autophagy-dependent metabolic reprogramming sensitizes TSC2-deficient cells to the antimetabolite 6-aminonicotinamide.
Neoplasms
Autophagy-dependent secretion: mechanism, factors secreted, and disease implications.
Neoplasms
B lymphoma Moloney murine leukemia virus insertion region 1: An oncogenic mediator in prostate cancer.
Neoplasms
BAY 87-2243, a novel inhibitor of hypoxia-induced gene activation, improves local tumor control after fractionated irradiation in a schedule-dependent manner in head and neck human xenografts.
Neoplasms
BCOR-coupled H2A monoubiquitination represses a subset of androgen receptor target genes regulating prostate cancer proliferation.
Neoplasms
Benign clear cell "sugar" tumor of the lung in a patient with Birt-Hogg-Dubé syndrome: a case report.
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Berberine as a Promising Safe Anti-Cancer Agent - Is there a Role for Mitochondria?
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Beyond rapalog therapy: preclinical pharmacology and antitumor activity of WYE-125132, an ATP-competitive and specific inhibitor of mTORC1 and mTORC2.
Neoplasms
Bio-active mixed ligand Cu(II) and Zn(II) complexes of pyrimidine derivative Schiff base: DFT calculation, antimicrobial, antioxidant, DNA binding, anticancer and molecular docking studies.
Neoplasms
Biochemical, cellular, and in vivo activity of novel ATP-competitive and selective inhibitors of the mammalian target of rapamycin.
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Biological evaluation of a cytotoxic 2-substituted benzimidazole copper(II) complex: DNA damage, antiproliferation and apoptotic induction activity in human cervical cancer cells.
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Bladder cancer cell-intrinsic PD-L1 signals promote mTOR and autophagy activation that can be inhibited to improve cytotoxic chemotherapy.
Neoplasms
Bmi1 enhances tumorigenicity and cancer stem cell function in pancreatic adenocarcinoma.
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BMI1 regulates PRC1 architecture and activity through homo- and hetero-oligomerization.
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c-Src kinase impairs the expression of mitochondrial OXPHOS complexes in liver cancer.
Neoplasms
C9orf72 and smcr8 mutant mice reveal MTORC1 activation due to impaired lysosomal degradation and exocytosis.
Neoplasms
Cachexia Disrupts Diurnal Regulation of Activity, Feeding, and Muscle Mechanistic Target of Rapamycin Complex 1 in Mice.
Neoplasms
Cancer cell specific inhibition of Wnt/?-catenin signaling by forced intracellular acidification.
Neoplasms
Carboxyamidotriazole inhibits oxidative phosphorylation in cancer cells and exerts synergistic anti-cancer effect with glycolysis inhibition.
Neoplasms
Causative role for defective expression of mitochondria-eating protein in accumulation of mitochondria in thyroid oncocytic cell tumors.
Neoplasms
CBX6 is negatively regulated by EZH2 and plays a potential tumor suppressor role in breast cancer.
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CBX7 and HMGA1b proteins act in opposite way on the regulation of the SPP1 gene expression.
Neoplasms
Celastrol targets mitochondrial respiratory chain complex I to induce reactive oxygen species-dependent cytotoxicity in tumor cells.
Neoplasms
Characterization of monoclonal antibodies against GRIM-19, a novel IFN-beta and retinoic acid-activated regulator of cell death.
Neoplasms
CHCHD4 regulates tumour proliferation and EMT-related phenotypes, through respiratory chain-mediated metabolism.
Neoplasms
Chemical genetics analysis of an aniline mustard anticancer agent reveals complex I of the electron transport chain as a target.
Neoplasms
Chromobox 4 facilitates tumorigenesis of lung adenocarcinoma through the Wnt/?-catenin pathway.
Neoplasms
Chromosomal localization of human GRIM-19, a novel IFN-beta and retinoic acid-activated regulator of cell death.
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Chronic activation of mTOR complex 1 is sufficient to cause hepatocellular carcinoma in mice.
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cis-[PtCl2(4,7-H-5-methyl-7-oxo[1,2,4]triazolo[1,5-a]pyrimidine)2]: a sterically restrictive new cisplatin analogue. Reaction kinetics with model nucleobases, DNA interaction studies, antitumor activity, and structure-activity relationships.
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Clinical and Histologic Analysis of the Efficacy of Topical Rapamycin Therapy Against Hypomelanotic Macules in Tuberous Sclerosis Complex.
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Clonal expansion of mutated mitochondrial DNA is associated with tumor formation and complex I deficiency in the benign renal oncocytoma.
Neoplasms
Colorectal Cancer and Mitochondrial Dysfunctions of the Adjunct Adipose Tissues: A Case Study.
Neoplasms
Comparison of the therapeutic effects of two vanadium complexes administered at low dose on benzo[a]pyrene-induced malignant tumors in rats.
Neoplasms
Complex I impairment in mitochondrial diseases and cancer: Parallel roads leading to different outcomes.
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Conditional disruption of rictor demonstrates a direct requirement for mTORC2 in skin tumor development and continued growth of established tumors.
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Construction and evaluation of a hncDNA library of human 12p transcribed sequences derived from a somatic cell hybrid.
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Control of B-cell lymphoma by therapeutic vaccination and acquisition of immune resistance is independent of direct tumour IFN-gamma signalling.
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Control of germline stem cell differentiation by polycomb and trithorax group genes in the niche microenvironment.
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Coordinate ?-adrenergic inhibition of mitochondrial activity and angiogenesis arrest tumor growth.
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Cordycepin as a sensitizer to tumour necrosis factor (TNF)-?-induced apoptosis through eukaryotic translation initiation factor 2? (eIF2?)- and mammalian target of rapamycin complex 1 (mTORC1)-mediated inhibition of nuclear factor (NF)-?B.
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Coupling mitochondrial respiratory chain to cell death: an essential role of mitochondrial complex I in the interferon-beta and retinoic acid-induced cancer cell death.
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Critical role of the tumor suppressor tuberous sclerosis complex 1 in dendritic cell activation of CD4 T cells by promoting MHC class II expression via IRF4 and CIITA.
Neoplasms
Cryptotanshinone activates AMPK-TSC2 axis leading to inhibition of mTORC1 signaling in cancer cells.
Neoplasms
Cubé resin insecticide: identification and biological activity of 29 rotenoid constituents.
Neoplasms
Cyclometalated Ruthenium(II) Anthraquinone Complexes Exhibit Strong Anticancer Activity in Hypoxic Tumor Cells.
Neoplasms
Cytotoxicity, dual-targeting apoptosis induction evaluation of multinuclear cu complexes based on pyrazine-benzimidazole derivative.
Neoplasms
Data mining of NCI's anticancer screening database reveals mitochondrial complex I inhibitors cytotoxic to leukemia cell lines.
Neoplasms
De novo prediction of cancer-associated T cell receptors for noninvasive cancer detection.
Neoplasms
Deconstructing feedback-signaling networks to improve anticancer therapy with mTORC1 inhibitors.
Neoplasms
Deconstructing mTOR complexes in regulation of Glioblastoma Multiforme and its stem cells.
Neoplasms
Decreased expression of GRIM-19 and its association with high-risk HPV infection in cervical squamous intraepithelial neoplasias and cancer.
Neoplasms
Deficiency of the complex I of the mitochondrial respiratory chain but improved adenylate control over succinate-dependent respiration are human gastric cancer-specific phenomena.
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Depletion of GRIM-19 accelerates hepatocellular carcinoma invasion via inducing EMT and loss of contact inhibition.
Neoplasms
Deregulated c-Myc requires a functional HSF1 for experimental and human hepatocarcinogenesis.
Neoplasms
Design, Synthesis, and Biological Features of Platinum(II) Complexes with Rigid Steric Hindrance.
Neoplasms
Detection of mitochondrial genome depletion by a novel cDNA in renal cell carcinoma.
Neoplasms
Development of MOF "Armor-Plated" Phycocyanin and Synergistic Inhibition of Cellular Respiration for Hypoxic Photodynamic Therapy in Patient-Derived Xenograft Models.
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Dichloroacetate enhances apoptotic cell death via oxidative damage and attenuates lactate production in metformin-treated breast cancer cells.
Neoplasms
Different mtDNA mutations modify tumor progression in dependence of the degree of respiratory complex I impairment.
Neoplasms
Differential inhibition/inactivation of mitochondrial complex I implicates its alteration in malignant cells.
Neoplasms
Differentially expressed mitochondrial genes in breast cancer cells: Potential new targets for anti-cancer therapies.
Neoplasms
Diorganotin (IV) complexes with 4-nitro-N-phthaloyl-glycine: Synthesis, characterization, antitumor activity and DNA-binding studies.
Neoplasms
Direct interaction of GD3 ganglioside with mitochondria generates reactive oxygen species followed by mitochondrial permeability transition, cytochrome c release, and caspase activation.
Neoplasms
Discovery Proteomics Identifies a Molecular Link between the Coatomer Protein Complex I and Androgen Receptor-dependent Transcription.
Neoplasms
Disproportionation and nuclease activity of bis[2-ethyl-2-hydroxybutanoato(2-)]oxochromate(V) in neutral aqueous solutions.
Neoplasms
Disruptive mitochondrial DNA mutations in complex I subunits are markers of oncocytic phenotype in thyroid tumors.
Neoplasms
Dissecting the expression landscape of mitochondrial genes in lung squamous cell carcinoma and lung adenocarcinoma.
Neoplasms
Dissociate expression of tuberous sclerosis complex 1 product hamartin in a skin and pulmonary lesion of a tuberous sclerosis complex.
Neoplasms
Distinct Ring1b complexes defined by DEAD-box helicases and EMT transcription factors synergistically enhance E-cadherin silencing in breast cancer.
Neoplasms
DNA cleavage, DNA/HSA binding study, and antiproliferative activity of a phenolate-bridged binuclear copper(II) complex.
Neoplasms
DNA-dependent protein kinase regulates lysosomal AMP-dependent protein kinase activation and autophagy.
Neoplasms
Down-regulation of GRIM-19 expression is associated with hyperactivation of STAT3-induced gene expression and tumor growth in human cervical cancers.
Neoplasms
Down-regulation of GRIM-19 promotes the growth and migration of human glioma cells.
Neoplasms
Down-Regulation of NDUFB9 Promotes Breast Cancer Cell Proliferation, Metastasis by Mediating Mitochondrial Metabolism.
Neoplasms
Downregulation of GRIM-19 is associated with hyperactivation of p-STAT3 in hepatocellular carcinoma.
Neoplasms
Downregulation of HSP60 disrupts mitochondrial proteostasis to promote tumorigenesis and progression in clear cell renal cell carcinoma.
Neoplasms
Doxorubicin-PAMAM dendrimer complex attached to liposomes: cytotoxic studies against human cancer cell lines.
Neoplasms
Dramatic suppression of colorectal cancer cell growth by the dual mTORC1 and mTORC2 inhibitor AZD-2014.
Neoplasms
Dual loss of succinate dehydrogenase (SDH) and complex I activity is necessary to recapitulate the metabolic phenotype of SDH mutant tumors.
Neoplasms
Dual mTORC1/2 inhibition in a preclinical xenograft tumor model of endometrial cancer.
Neoplasms
Dual mTORC1/mTORC2 inhibition diminishes Akt activation and induces Puma-dependent apoptosis in lymphoid malignancies.
Neoplasms
Dual Roles of Nicotinamide Phosphoribosyltransferase as a Promising Target for Cancer Radiotherapy.
Neoplasms
Dual targeting of the cancer antioxidant network with 1,4-naphthoquinone fused Gold(i) N-heterocyclic carbene complexes.
Neoplasms
Dynamic Imaging of LDH Inhibition in Tumors Reveals Rapid In Vivo Metabolic Rewiring and Vulnerability to Combination Therapy.
Neoplasms
Dynamin-dependent amino acid endocytosis activates mechanistic target of rapamycin complex 1 (mTORC1).
Neoplasms
Early loss of mitochondrial complex I and rewiring of glutathione metabolism in renal oncocytoma.
Neoplasms
Effect of Dactylogyrus catlaius (Jain 1961) infection in Labeo rohita (Hamilton 1822): innate immune responses and expression profile of some immune related genes.
Neoplasms
Effects of new tetrahydroquinoline-isoxazole hybrides on bioenergetics of hepatocarcinoma Hep-G2 cells and rat liver mitochondria.
Neoplasms
Effects of plasmid-based Stat3-specific short hairpin RNA and GRIM-19 on PC-3M tumor cell growth.
Neoplasms
Effects of single-nucleotide polymorphisms in the mTORC1 pathway on the risk of brain metastasis in patients with non-small cell lung cancer.
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Efficacy of Ganitumab (AMG 479), Alone and in Combination With Rapamycin, in Ewing's and Osteogenic Sarcoma Models.
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EGFR signaling through an Akt-SREBP-1-dependent, rapamycin-resistant pathway sensitizes glioblastomas to antilipogenic therapy.
Neoplasms
Electron-transferring enzymes in the plasma membrane of the Ehrlich ascites tumor cell.
Neoplasms
Endoplasmic reticulum stress and cell death in mTORC1-overactive cells is induced by nelfinavir and enhanced by chloroquine.
Neoplasms
Energy metabolism modulation by biguanides in comparison with rotenone in rat liver and heart.
Neoplasms
Engineered Bacterial Bioreactor for Tumor Therapy via Fenton-Like Reaction with Localized H2 O2 Generation.
Neoplasms
Enhanced mLST8 Expression Correlates with Tumor Progression in Hepatocellular Carcinoma.
Neoplasms
Enhanced Sensitivity of Nonsmall Cell Lung Cancer with Acquired Resistance to Epidermal Growth Factor Receptor-Tyrosine Kinase Inhibitors to Phenformin: The Roles of a Metabolic Shift to Oxidative Phosphorylation and Redox Balance.
Neoplasms
Environment Dictates Dependence on Mitochondrial Complex I for NAD+ and Aspartate Production and Determines Cancer Cell Sensitivity to Metformin.
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Epigenetic regulation of survivin by Bmi1 is cell type specific during corticogenesis and in gliomas.
Neoplasms
Evaluation of cancer testis antigen (CT10, PRAME) and MHC I expression in high-grade urothelial carcinoma of the bladder.
Neoplasms
Evidence for a plasma membrane redox system on intact ascites tumor cells with different metastatic capacity.
Neoplasms
EVT-701 is a novel selective and safe mitochondrial complex 1 inhibitor with potent anti-tumor activity in models of solid cancers.
Neoplasms
Exploring a glycolytic inhibitor for the treatment of an FH-deficient type-2 papillary RCC.
Neoplasms
Expression of total and phospho 4EBP1 in metastatic and non-metastatic renal cell carcinoma.
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Ferrocenyl-Triphenyltin Complexes as Lysosome-Targeted Imaging and Anticancer Agents.
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First Synthesis of an Oridonin-Conjugated Iridium(III) Complex for the Intracellular Tracking of NF-?B in Living Cells.
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First-in-human evaluation of the novel mitochondrial complex I inhibitor ASP4132 for treatment of cancer.
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Flura-seq identifies organ-specific metabolic adaptations during early metastatic colonization.
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Frequency and phenotypic implications of mitochondrial DNA mutations in human squamous cell cancers of the head and neck.
Neoplasms
Gene associated with retinoid-interferon-induced mortality-19 suppresses growth of lung adenocarcinoma tumor in vitro and in vivo.
Neoplasms
Generation of trans-mitochondrial mito-mice by the introduction of a pathogenic G13997A mtDNA from highly metastatic lung carcinoma cells.
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Genetic variations of mTORC1 genes and risk of gastric cancer in an Eastern Chinese population.
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Genome-wide RNA interference screening reveals a COPI-MAP2K3 pathway required for YAP regulation.
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Genomics of chromophobe renal cell carcinoma: implications from a rare tumor for pan-cancer studies.
Neoplasms
Glutaminolysis is involved in the activation of mTORC1 in in vitro-produced porcine embryos.
Neoplasms
GRIM-19 and p16(INK4a) synergistically regulate cell cycle progression and E2F1-responsive gene expression.
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GRIM-19 deficiency promotes clear cell renal cell carcinoma progression and is associated with high TNM stage and Fuhrman grade.
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GRIM-19 Disrupts E6/E6AP Complex to Rescue p53 and Induce Apoptosis in Cervical Cancers.
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Grim-19 expression and its close association with tumor progression in systemic malignancies.
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GRIM-19 inhibition induced autophagy through activation of ERK and HIF-1? not STAT3 in Hela cells.
Neoplasms
GRIM-19 inhibits v-Src-induced cell motility by interfering with cytoskeletal restructuring.
Neoplasms
GRIM-19 opposes reprogramming of glioblastoma cell metabolism via HIF1? destabilization.
Neoplasms
GRIM-19 over-expression represses the proliferation and invasion of orthotopically implanted hepatocarcinoma tumors associated with downregulation of Stat3 signaling.
Neoplasms
GRIM-19 represses the proliferation and invasion of cutaneous squamous cell carcinoma cells associated with downregulation of STAT3 signaling.
Neoplasms
GRIM-19 Restores Cervical Cancer Cell Senescence by Repressing hTERT Transcription.
Neoplasms
GRIM-19, a cell death regulatory protein, is essential for assembly and function of mitochondrial complex I.
Neoplasms
GRIM-19, a death-regulatory gene product, suppresses Stat3 activity via functional interaction.
Neoplasms
GRIM-19: A master regulator of cytokine induced tumor suppression, metastasis and energy metabolism.
Neoplasms
Guanaconetins, new antitumoral acetogenins, mitochondrial complex I and tumor cell growth inhibitors.
Neoplasms
Half-Sandwich Ru(p-cymene) Compounds with Diphosphanes: In Vitro and In Vivo Evaluation As Potential Anticancer Metallodrugs.
Neoplasms
Heightening Energetic Stress Selectively Targets LKB1-Deficient Non-Small Cell Lung Cancers.
Neoplasms
Hepatic mTORC1 controls locomotor activity, body temperature, and lipid metabolism through FGF21.
Neoplasms
Heteroleptic Copper(I) Complexes of "Scorpionate" Bis-pyrazolyl Carboxylate Ligand with Auxiliary Phosphine as Potential Anticancer Agents: An Insight into Cytotoxic Mode.
Neoplasms
High frequency of somatic mitochondrial DNA mutations in human thyroid carcinomas and complex I respiratory defect in thyroid cancer cell lines.
Neoplasms
High Glucose Induces Down-Regulated GRIM-19 Expression to Activate STAT3 Signaling and Promote Cell Proliferation in Cell Culture.
Neoplasms
High in vitro and in vivo antitumor activities of Ln(III) complexes with mixed 5,7-dichloro-2-methyl-8-quinolinol and 4,4'-dimethyl-2,2'-bipyridyl chelating ligands.
Neoplasms
High-dose rapamycin induces apoptosis in human cancer cells by dissociating mTOR complex 1 and suppressing phosphorylation of 4E-BP1.
Neoplasms
Highly Promising Antitumor Agent of a Novel Platinum(II) Complex Bearing a Tetradentate Chelating Ligand.
Neoplasms
HIV-1 TAT-mediated microglial activation: role of mitochondrial dysfunction and defective mitophagy.
Neoplasms
Human TSC2-null fibroblast-like cells induce hair follicle neogenesis and hamartoma morphogenesis.
Neoplasms
Hyperactivated mTORC1 downregulation of FOXO3a/PDGFR?/AKT cascade restrains tuberous sclerosis complex-associated tumor development.
Neoplasms
Hypoxia induces a phase transition within a kinase signaling network in cancer cells.
Neoplasms
Hypoxia-inducible factor pathway inhibition resolves tumor hypoxia and improves local tumor control after single-dose irradiation.
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IACS-010759, a potent inhibitor of glycolysis-deficient hypoxic tumor cells, inhibits mitochondrial respiratory complex I through a unique mechanism.
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Identification of a novel toxicophore in anti-cancer chemotherapeutics that targets mitochondrial respiratory complex I.
Neoplasms
Identification of an iridium(III) complex with anti-bacterial and anti-cancer activity.
Neoplasms
Identification of collaborative activities with oxidative phosphorylation in bipolar disorder.
Neoplasms
Identification of human renal cell carcinoma associated genes by suppression subtractive hybridization.
Neoplasms
Identification of key genes in Gram?positive and Gram?negative sepsis using stochastic perturbation.
Neoplasms
Identification of novel cancer therapeutic targets using a designed and pooled shRNA library screen.
Neoplasms
Identification of potential drug targets for tuberous sclerosis complex by synthetic screens combining CRISPR-based knockouts with RNAi.
Neoplasms
Identification of proteins suppressing the functions of oncogenic phosphatase of regenerating liver 1 and 3.
Neoplasms
Identifying Mitochondrial-Related Genes NDUFA10 and NDUFV2 as Prognostic Markers for Prostate Cancer through Biclustering.
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Immune/Hypoxic Tumor Microenvironment Regulation-Enhanced Photodynamic Treatment Realized by pH-Responsive Phase Transition-Targeting Nanobubbles.
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Immunizations with IFNgamma secreting tumor cells can eliminate fully established and invasive rat gliomas.
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Immunohistochemical Assessment of Phosphorylated mTORC1-Pathway Proteins in Human Brain Tumors.
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Improved efficacy of mitochondrial disrupting agents upon inhibition of autophagy in a mouse model of BRCA1-deficient breast cancer.
Neoplasms
In vitro antitumor structure-activity relationships of threo/trans/threo/trans/erythro bis-tetrahydrofuranic acetogenins: correlations with their inhibition of mitochondrial complex I.
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In vitro growth environment produces lipidomic and electron transport chain abnormalities in mitochondria from non-tumorigenic astrocytes and brain tumours.
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Increase in proteins involved in mitochondrial fission, mitophagy, proteolysis and antioxidant response in type I endometrial cancer as an adaptive response to respiratory complex I deficiency.
Neoplasms
Inducing cancer indolence by targeting mitochondrial Complex I is potentiated by blocking macrophage-mediated adaptive responses.
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Induction of cell death of gastric cancer cells by a modified compound of the annonaceous acetogenin family.
Neoplasms
Induction of cytotoxicity against autologous tumour cells by interleukin-12: evidence for intrinsic anti-tumor immune capacity in curatively resected gastrointestinal tumour patients.
Neoplasms
Inhibition of mitochondrial respiration prevents BRAF-mutant melanoma brain metastasis.
Neoplasms
Inhibition of monocarboxyate transporter 1 by AZD3965 as a novel therapeutic approach for diffuse large B-cell lymphoma and Burkitt lymphoma.
Neoplasms
Inhibition of mTOR complex 1/p70 S6 kinase signaling elevates PD-L1 levels in human cancer cells through enhancing protein stabilization accompanied with enhanced ?-TrCP degradation.
Neoplasms
Inhibition of NF-?B nuclear translocation via HO-1 activation underlies ?-tocopheryl succinate toxicity.
Neoplasms
Inhibition of the mTORC1 pathway suppresses intestinal polyp formation and reduces mortality in ApcDelta716 mice.
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Inhibitory effect of nordihydroguaiaretic acid and its tetra-acetylated derivative on respiration and growth of adenocarcinoma TA3 and its multiresistant variant TA3MTX-R.
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Inositol polyphosphate multikinase is a coactivator for serum response factor-dependent induction of immediate early genes.
Neoplasms
Insights into the cytotoxic activity of the phosphane copper(I) complex [Cu(thp)4][PF6].
Neoplasms
Integration of gene expression profile data to screen and verify immune-related genes of chicken erythrocytes involved in Marek's disease virus.
Neoplasms
Interleukin-7 gene transfer in non-small-cell lung cancer decreases tumor proliferation, modifies cell surface molecule expression, and enhances antitumor reactivity.
Neoplasms
Involvement of p53 in cell death following cell cycle arrest and mitotic catastrophe induced by rotenone.
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JNK Signaling Promotes Intestinal Tumorigenesis Through Activation of mTOR Complex 1 in Apc?716 Mice.
Neoplasms
K-Ras mutation-mediated IGF-1-induced feedback ERK activation contributes to the rapalog resistance in pancreatic ductal adenocarcinomas.
Neoplasms
Kaposi sarcoma-associated herpesvirus miRNAs suppress CASTOR1-mediated mTORC1 inhibition to promote tumorigenesis.
Neoplasms
KIAA1524/CIP2A promotes cancer growth by coordinating the activities of MTORC1 and MYC.
Neoplasms
KICSTOR recruits GATOR1 to the lysosome and is necessary for nutrients to regulate mTORC1.
Neoplasms
Kinetic Modeling and Analysis of the Akt/Mechanistic Target of Rapamycin Complex 1 (mTORC1) Signaling Axis Reveals Cooperative, Feedforward Regulation.
Neoplasms
Knockdown BMI1 expression inhibits proliferation and invasion in human bladder cancer T24 cells.
Neoplasms
Knockdown of THOC1 reduces the proliferation of hepatocellular carcinoma and increases the sensitivity to cisplatin.
Neoplasms
Lethal Poisoning of Cancer Cells by Respiratory Chain Inhibition plus Dimethyl ?-Ketoglutarate.
Neoplasms
Levels of 4EBP1/eIF4E Activation in Renal Cell Carcinoma Could Differentially Predict Its Early and Late Recurrence.
Neoplasms
Linagliptin potentiates the effect of l-dopa on the behavioural, biochemical and immunohistochemical changes in experimentally-induced Parkinsonism: Role of toll-like receptor 4, TGF-?1, NF-?B and glucagon-like peptide 1.
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LncRNA-MEG3 inhibits cell proliferation and invasion by modulating Bmi1/RNF2 in cholangiocarcinoma.
Neoplasms
Loss of polycomb repressive complex 1 activity and chromosomal instability drive uveal melanoma progression.
Neoplasms
Loss of TSC complex enhances gluconeogenesis via upregulation of Dlk1-Dio3 locus miRNAs.
Neoplasms
Loss of tuberous sclerosis complex 2 sensitizes tumors to nelfinavir-bortezomib therapy to intensify endoplasmic reticulum stress-induced cell death.
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Low VDAC1 Expression Is Associated with an Aggressive Phenotype and Reduced Overall Patient Survival in Cholangiocellular Carcinoma.
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Luminescent iridium(iii) complexes as COX-2-specific imaging agents in cancer cells.
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Lymphangioleiomyomatosis Biomarkers Linked to Lung Metastatic Potential and Cell Stemness.
Neoplasms
LYRM2 directly regulates complex I activity to support tumor growth in colorectal cancer by oxidative phosphorylation.
Neoplasms
Macrophage Raptor Deficiency-Induced Lysosome Dysfunction Exacerbates Nonalcoholic Steatohepatitis.
Neoplasms
Major histocompatibility complex I upregulation in clear cell renal cell carcinoma is associated with increased survival.
Neoplasms
Malignant manipulaTORs of metabolism: suppressing BCAA catabolism to enhance mTORC1 activity.
Neoplasms
Mammalian NADH:ubiquinone oxidoreductase (Complex I) and nicotinamide nucleotide transhydrogenase (Nnt) together regulate the mitochondrial production of H?O?--implications for their role in disease, especially cancer.
Neoplasms
Manipulation of tumor metabolism for therapeutic approaches: ovarian cancer-derived cell lines as a model system.
Neoplasms
MCT4 is induced by metastasis-enhancing pathogenic mitochondrial NADH dehydrogenase gene mutations and can be a therapeutic target.
Neoplasms
Mechanism-based modeling of the clinical effects of bevacizumab and everolimus on vestibular schwannomas of patients with neurofibromatosis type 2.
Neoplasms
Mechanistic Investigations of the Mitochondrial Complex I Inhibitor Rotenone in the Context of Pharmacological and Safety Evaluation.
Neoplasms
Metabolic control analysis indicates a change of strategy in the treatment of cancer.
Neoplasms
Metabolic determinants of cancer cell sensitivity to glucose limitation and biguanides.
Neoplasms
Metabolic reprogramming toward oxidative phosphorylation identifies a therapeutic target for mantle cell lymphoma.
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Metformin Antagonizes Cancer Cell Proliferation by Suppressing Mitochondrial-Dependent Biosynthesis.
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Metformin Clinical Trial in HPV+ and HPV- Head and Neck Squamous Cell Carcinoma: Impact on Cancer Cell Apoptosis and Immune Infiltrate.
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Metformin inhibits mitochondrial complex I of cancer cells to reduce tumorigenesis.
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Metformin Inhibits Progression of Head and Neck Squamous Cell Carcinoma by Acting Directly on Carcinoma-Initiating Cells.
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Metformin-Induced Mitochondrial Complex I Inhibition: Facts, Uncertainties, and Consequences.
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MicroRNA-141-3p regulates cellular proliferation, migration, and invasion in esophageal cancer by targeting tuberous sclerosis complex 1.
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Minimization of energy transduction confers resistance to phosphine in the rice weevil, Sitophilus oryzae.
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Mitochondria and redox homoeostasis as chemotherapeutic targets of Araucaria angustifolia (Bert.) O. Kuntze in human larynx HEp-2 cancer cells.
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Mitochondria-localizing dicarbohydrazide Ln complexes and their mechanism of in vitro anticancer activity.
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Mitochondrial biogenesis in epithelial cancer cells promotes breast cancer tumor growth and confers autophagy resistance.
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Mitochondrial complex I activity and NAD+/NADH balance regulate breast cancer progression.
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Mitochondrial complex I inhibition triggers a mitophagy-dependent ROS increase leading to necroptosis and ferroptosis in melanoma cells.
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Mitochondrial complex I inhibitors and forced oxidative phosphorylation synergize in inducing cancer cell death.
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Mitochondrial DNA mutations distinguish bilateral multifocal renal oncocytomas from familial Birt-Hogg-Dubé tumors.
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Mitochondrial DNA mutations induce mitochondrial biogenesis and increase the tumorigenic potential of Hodgkin and Reed-Sternberg cells.
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Mitochondrial DNA polymorphism in genes encoding ND1, COI and CYTB in canine malignant cancers.
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Mitochondrial DNA somatic mutations (point mutations and large deletions) and mitochondrial DNA variants in human thyroid pathology: a study with emphasis on Hürthle cell tumors.
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Mitochondrial dysfunction and therapeutic approaches in respiratory and limb muscles of cancer cachectic mice.
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Mitochondrial dysfunction induces radioresistance in colorectal cancer by activating [Ca2+]m-PDP1-PDH-histone acetylation retrograde signaling.
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Mitochondrial dysfunction promotes breast cancer cell migration and invasion through HIF1? accumulation via increased production of reactive oxygen species.
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Mitochondrial Dysfunctions in Type I Endometrial Carcinoma: Exploring Their Role in Oncogenesis and Tumor Progression.
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Mitochondrial electron-transport-chain inhibitors of complexes I and II induce autophagic cell death mediated by reactive oxygen species.
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Mitochondrial GRIM-19 as a potential therapeutic target for STAT3-dependent carcinogenesis of gastric cancer.
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Mitochondrial metabolic reprogramming controls the induction of immunogenic cell death and efficacy of chemotherapy in bladder cancer.
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Mitochondrial ND5 mutation mediated elevated ROS regulates apoptotic pathway epigenetically in a P53 dependent manner for generating pro-cancerous phenotypes.
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Mitochondrial Oxidative Stress due to Complex I Dysfunction Promotes Fibroblast Activation and Melanoma Cell Invasiveness.
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Mitochondrial Respiration in Human Colorectal and Breast Cancer Clinical Material Is Regulated Differently.
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Mitochondrial-Localized Stat3 Promotes Breast Cancer Growth via Phosphorylation of Serine 727.
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Modified Metformin as a More Potent Anticancer Drug: Mitochondrial Inhibition, Redox Signaling, Antiproliferative Effects and Future EPR Studies.
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Molecular phylogenetics unveils the ancient evolutionary origins of the enigmatic fairy armadillos.
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Molecular signal networks and regulating mechanisms of the unfolded protein response.
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Molecular-Targeted Antitumor Agents. 19. Furospongolide from a Marine Lendenfeldia sp. Sponge Inhibits Hypoxia-Inducible Factor-1 Activation in Breast Tumor Cells.
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mTOR and HIF-1alpha-mediated tumor metabolism in an LKB1 mouse model of Peutz-Jeghers syndrome.
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mTOR, a new potential target for chronic pain and opioid-induced tolerance and hyperalgesia.
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mTOR-targeted cancer therapy: great target but disappointing clinical outcomes, why?
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mTORC1 inhibitors: is temsirolimus in renal cancer telling us how they really work?
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mTORC1 upregulation via ERK-dependent gene expression change confers intrinsic resistance to MEK inhibitors in oncogenic KRas-mutant cancer cells.
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mTORC1-mediated downregulation of COX2 restrains tumor growth caused by TSC2 deficiency.
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mTORC2 Balances AKT Activation and eIF2? Serine 51 Phosphorylation to Promote Survival under Stress.
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Mutant ras elevates dependence on serum lipids and creates a synthetic lethality for rapamycin.
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Mutation in mitochondrial complex I ND6 subunit is associated with defective response to hypoxia in human glioma cells.
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Mutation of the co-chaperone Tsc1 in bladder cancer diminishes Hsp90 acetylation and reduces drug sensitivity and selectivity.
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Mutations in the ND2 Subunit of Mitochondrial Complex I Are Sufficient to Confer Increased Tumorigenic and Metastatic Potential to Cancer Cells.
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N-Formyl-3,4-methylenedioxy-benzylidene-gamma-butyrolaetam, KNK437 induces caspase-3 activation through inhibition of mTORC1 activity in Cos-1 cells.
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NADH dehydrogenase complex I is overexpressed in incipient metastatic murine colon cancer cells.
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NDUFA4L2 promotes glioblastoma progression, is associated with poor survival, and can be effectively targeted by apatinib.
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New Amino Acid Schiff Bases as Anticancer Agents via Potential Mitochondrial Complex I-Associated Hexokinase Inhibition and Targeting AMP-Protein Kinases/mTOR Signaling Pathway.
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New cyclometalated gold (III) complex targeting thioredoxin reductase: exploring as cytotoxic agents and mechanistic insights.
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New Multinuclear Scaffold Molybdocene-Gold Lidocaine Complex: DNA/HSA Binding, Molecular Docking, Cytotoxicity and Mechanistic Insights.
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NF2/merlin is a novel negative regulator of mTOR complex 1, and activation of mTORC1 is associated with meningioma and schwannoma growth.
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Nicotinamide phosphoribosyltransferase can affect metastatic activity and cell adhesive functions by regulating integrins in breast cancer.
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Nitroxide TEMPOL impairs mitochondrial function and induces apoptosis in HL60 cells.
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Nonsense and missense mutation of mitochondrial ND6 gene promotes cell migration and invasion in human lung adenocarcinoma.
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Nonviral Delivery of GRIM-19 Gene Inhibits Tumor Growth with Reduced Local and Systemic Complications.
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Novel binuclear and trinuclear metal (II) complexes: DNA interactions and in vitro anticancer activity through apoptosis.
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Novel mononuclear Cu (II) terpyridine complexes: Impact of fused ring thiophene and thiazole head groups towards DNA/BSA interaction, cleavage and antiproliferative activity on HepG2 and triple negative CAL-51 cell line.
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Novel ruthenium(II) triazine complex [Ru(bdpta)(tpy)]2+ co-targeting drug resistant GRP78 and subcellular organelles in cancer stem cells.
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Nucleoporin TPR (translocated promoter region, nuclear basket protein) upregulation alters MTOR-HSF1 trails and suppresses autophagy induction in ependymoma.
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Nudix-type motif 2 contributes to cancer proliferation through the regulation of Rag GTPase-mediated mammalian target of rapamycin complex 1 localization.
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Octahedral copper(ii)-diimine complexes of triethylenetetramine: effect of stereochemical fluxionality and ligand hydrophobicity on CuII/CuI redox, DNA binding and cleavage, cytotoxicity and apoptosis-inducing ability.
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Olfactomedin 4 promotes S-phase transition in proliferation of pancreatic cancer cells.
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Oncocytic tumors are marked by enhanced mitochondrial content and mtDNA mutations of complex I in Chinese patients.
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Overexpression of GRIM-19 in cancer cells suppresses STAT3-mediated signal transduction and cancer growth.
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Overexpression of GRIM-19, a mitochondrial respiratory chain complex I protein, suppresses hepatocellular carcinoma growth.
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Overexpression of Lon contributes to survival and aggressive phenotype of cancer cells through mitochondrial complex I-mediated generation of reactive oxygen species.
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Overexpression of NDUFA4L2 is associated with poor prognosis in patients with colorectal cancer.
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Overexpression of RNF2 is positively associated with ovarian carcinoma aggressiveness and indicative of poor patient survival.
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Oxidative stress-mediated cytotoxicity of Endosulfan is causally linked to the inhibition of NADH dehydrogenase and Na+, K+-ATPase in Ehrlich ascites tumor cells.
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Oxovanadium(IV) complexes of curcumin for cellular imaging and mitochondria targeted photocytotoxicity.
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p53 increases MHC class I expression by upregulating the endoplasmic reticulum aminopeptidase ERAP1.
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Pharmacological inhibition of Polo-like kinase 1 (PLK1) by BI-2536 decreases the viability and survival of hamartin and tuberin deficient cells via induction of apoptosis and attenuation of autophagy.
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Phase II study of everolimus in children and adults with neurofibromatosis type 2 and progressive vestibular schwannomas.
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Phase II study of mTORC1 inhibition by everolimus in neurofibromatosis type 2 patients with growing vestibular schwannomas.
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Phenformin-Induced Mitochondrial Dysfunction Sensitizes Hepatocellular Carcinoma for Dual Inhibition of mTOR.
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Phosphatase-like activity, DNA binding, DNA hydrolysis, anticancer and lactate dehydrogenase inhibition activity promoting by a new bis-phenanthroline dicopper(II) complex.
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Phosphatidylinositol 3,4-bisphosphate synthesis and turnover are spatially segregated in the endocytic pathway.
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Photoactivatable diazido Pt(IV) anticancer complex can bind to and oxidize all four nucleosides.
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Physical Training Regulates Mitochondrial Parameters and Neuroinflammatory Mechanisms in an Experimental Model of Parkinson's Disease.
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Phytocannabinoid-dependent mTORC1 regulation is dependent upon inositol polyphosphate multikinase activity.
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Plasmid-based Stat3-specific siRNA and GRIM-19 inhibit the growth of thyroid cancer cells in vitro and in vivo.
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Plasmid-based Survivin shRNA and GRIM-19 carried by attenuated Salmonella suppresses tumor cell growth.
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Platinum(II) Complexes with 1,10-Phenanthroline and Hydrophilic Alkoxyacetate Ligands as Potential Antitumor Agents.
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Platinum(iv) dihydroxido diazido N-(heterocyclic)imine complexes are potently photocytotoxic when irradiated with visible light.
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Polycomb group molecule PHC3 regulates polycomb complex composition and prognosis of osteosarcoma.
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Polycomb repressor complex 1 promotes gene silencing through H2AK119 mono-ubiquitination in acinar-to-ductal metaplasia and pancreatic cancer cells.
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Potent inhibition of tumour cell proliferation and immunoregulatory function by mitochondria-targeted atovaquone.
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PP242 suppresses bladder cancer cell proliferation and migration through deactivating the mammalian target of rapamycin complex 2/AKT1 signaling pathway.
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Preservation of NADH ubiquinone-oxidoreductase activity by Src kinase-mediated phosphorylation of NDUFB10.
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Prevention of alveolar destruction and airspace enlargement in a mouse model of pulmonary lymphangioleiomyomatosis (LAM).
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Prognostic significance of mitochondrial oxidative phosphorylation complexes: Therapeutic target in the treatment of retinoblastoma.
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Proteomic analysis of p38alpha mitogen-activated protein kinase-regulated changes in membrane fractions of RAS-transformed fibroblasts.
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Proton-assisted amino-acid transporters are conserved regulators of proliferation and amino-acid-dependent mTORC1 activation.
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Purification and characterization of a plasma membrane ferricyanide-utilizing NADH dehydrogenase from Ehrlich tumour cells.
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Radical-induced DNA damage by cytotoxic square-planar copper(II) complexes incorporating o-phthalate and 1,10-phenanthroline or 2,2'-dipyridyl.
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Rap1-GTPases control mTORC1 activity by coordinating lysosome organization with amino acid availability.
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Rapamycin Prevents Surgery-Induced Immune Dysfunction in Patients with Bladder Cancer.
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Rapid induction of apoptosis in tumor cells treated with a new platinum(II) complex based on amino-thiazolidinone.
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RAPTOR promotes colorectal cancer proliferation by inducing mTORC1 and upregulating ribosome assembly factor URB1.
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Redox state of adipose tissue for patients with gastric cancer and its connection with the body mass index and distance from the tumor.
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Reduced Levels of ATP Synthase Subunit ATP5F1A Correlate with Earlier-Onset Prostate Cancer.
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Regulation of mammalian target of rapamycin complex 1 (mTORC1) by hypoxia: causes and consequences.
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Regulation of mTOR complex 2 signaling in neurofibromatosis 2-deficient target cell types.
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Regulation of tumor necrosis factor-induced, mitochondria- and reactive oxygen species-dependent cell death by the electron flux through the electron transport chain complex I.
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Relationship between metabolism and peripheral blood mononuclear cell mitochondrial complex I activity before and after a short-term refeeding in weight-losing cancer patients.
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Renal oncocytoma characterized by the defective complex I of the respiratory chain boosts the synthesis of the ROS scavenger glutathione.
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Renal tumours in a Tsc2(+/-) mouse model do not show feedback inhibition of Akt and are effectively prevented by rapamycin.
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Reprogramming metabolism with metformin improves tumor oxygenation and radiotherapy response.
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Respiratory chain complex I is a mitochondrial tumor suppressor of oncocytic tumors.
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Respiratory complex I is essential to induce a Warburg profile in mitochondria-defective tumor cells.
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Response to everolimus is seen in TSC-associated SEGAs and angiomyolipomas independent of mutation type and site in TSC1 and TSC2.
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Resveratrol prevents rapamycin-induced upregulation of autophagy and selectively induces apoptosis in TSC2-deficient cells.
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Role of I?B kinase-? in the growth-promoting effects of angiotensin II in vitro and in vivo.
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Rotenone induces apoptosis in human lung cancer cells by regulating autophagic flux.
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Royal jelly attenuates metabolic defects in a Drosophila mutant with elevated TORC1 activity.
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Ru(II)-Based Amino Acid Complexes Show Promise for Leukemia Treatment: Cytotoxicity and Some Light on their Mechanism of Action.
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Ruthenium Complexes With Piplartine Cause Apoptosis Through MAPK Signaling by a p53-Dependent Pathway in Human Colon Carcinoma Cells and Inhibit Tumor Development in a Xenograft Model.
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Ruthenium(II) p-cymene complex bearing 2,2'-dipyridylamine targets caspase 3 deficient MCF-7 breast cancer cells without disruption of antitumor immune response.
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Ruthenium(II) salicylate complexes inducing ROS-mediated apoptosis by targeting thioredoxin reductase.
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S100A4 alters metabolism and promotes invasion of lung cancer cells by up-regulating mitochondrial complex I protein NDUFS2.
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S6 kinase 1 is required for rapamycin-sensitive liver proliferation after mouse hepatectomy.
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S6K1 regulates GSK3 under conditions of mTOR-dependent feedback inhibition of Akt.
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Seeking mTORC1 Inhibitors Through Molecular Dynamics Simulation of Arginine Analogs Inhibiting CASTOR1.
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Selected gene profiles of stressed NSC-34 cells and rat spinal cord following peripheral nerve reconstruction and minocycline treatment.
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Selective Inhibition of Lysine-Specific Demethylase 5A (KDM5A) Using a Rhodium(III) Complex for Triple-Negative Breast Cancer Therapy.
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Selective inhibition of mitochondrial respiration and glycolysis in human leukaemic leucocytes by methylglyoxal.
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Self-assembled Cu(II) and Ni(II) metallamacrocycles formed from 3,3,3',3'-tetrabenzyl-1,1'-aroylbis(thiourea) ligands: DNA and protein binding studies, and cytotoxicity of trinuclear complexes.
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Senescence Induced by BMI1 Inhibition Is a Therapeutic Vulnerability in H3K27M-Mutant DIPG.
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Serine 302 Phosphorylation of Mouse Insulin Receptor Substrate 1 (IRS1) Is Dispensable for Normal Insulin Signaling and Feedback Regulation by Hepatic S6 Kinase.
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Serum lactate dehydrogenase predicts for overall survival benefit in patients with metastatic renal cell carcinoma treated with inhibition of Mammalian target of rapamycin.
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Several distinct polycomb complexes regulate and co-localize on the INK4a tumor suppressor locus.
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Significance of low mTORC1 activity in defining the characteristics of brain tumor stem cells.
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Significant prognostic values of nuclear genes encoding mitochondrial complex I subunits in tumor patients.
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SiO? nanoparticle-induced impairment of mitochondrial energy metabolism in hepatocytes directly and through a Kupffer cell-mediated pathway in vitro.
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SIRT3 aggravates metformin-induced energy stress and apoptosis in ovarian cancer cells.
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Somatic and germline mutation in GRIM-19, a dual function gene involved in mitochondrial metabolism and cell death, is linked to mitochondrion-rich (Hurthle cell) tumours of the thyroid.
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Somatic complex I disruptive mitochondrial DNA mutations are modifiers of tumorigenesis that correlate with low genomic instability in pituitary adenomas.
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Somatic mitochondrial DNA mutations in cancer escape purifying selection and high pathogenicity mutations lead to the oncocytic phenotype: pathogenicity analysis of reported somatic mtDNA mutations in tumors.
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Somatic Mutations of TSC2 or MTOR Characterize a Morphologically Distinct Subset of Sporadic Renal Cell Carcinoma With Eosinophilic and Vacuolated Cytoplasm.
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Specific disruption of Tsc1 in ovarian granulosa cells promotes ovulation and causes progressive accumulation of corpora lutea.
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Spreading of mesothelioma cells is rapamycin-sensitive and requires continuing translation.
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Stabilization of G-quadruplex DNA and inhibition of telomerase activity studies of ruthenium(II) complexes.
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STAT3 inhibition induced temozolomide-resistant glioblastoma apoptosis via triggering mitochondrial STAT3 translocation and respiratory chain dysfunction.
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STAT3 Inhibitor OPB-51602 Is Cytotoxic to Tumor Cells Through Inhibition of Complex I and ROS Induction.
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Structural mechanism of a Rag GTPase activation checkpoint by the lysosomal folliculin complex.
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Structure elucidation {spectroscopic, single crystal X-ray diffraction and computational DFT studies} of new tailored benzenesulfonamide derived Schiff base copper(II) intercalating complexes: Comprehensive biological profile {DNA binding, pBR322 DNA cleavage, Topo I inhibition and cytotoxic activity}.
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Structure of the human mTOR complex I and its implications for rapamycin inhibition.
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Structure, cytotoxicity, and DNA-cleavage properties of the complex [Cu(II)(pbt)Br2].
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Structure-activity analysis of niclosamide reveals potential role for cytoplasmic pH in control of mammalian target of rapamycin complex 1 (mTORC1) signaling.
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Structure-activity relationships of hybrid annonaceous acetogenins: powerful growth inhibitory effects of their connecting groups between heterocycle and hydrophobic carbon chain bearing THF ring on human cancer cell lines.
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Structure-Guided Discovery of Selective Antagonists for the Chromodomain of Polycomb Repressive Protein CBX7.
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Studies on characterization, telomerase inhibitory properties and G-quadruplex binding of ?6-arene ruthenium complexes with 1,10-phenanthroline-derived ligands.
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Suppression of mTOR complex 2-dependent AKT phosphorylation in melanoma cells by combined treatment with rapamycin and LY294002.
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Suppression of mTOR via Akt dependent and independent mechanisms in selenium treated colon cancer cells: involvement of AMPK{alpha}1.
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SWATH-MS proteomics of PANC-1 and MIA PaCa-2 pancreatic cancer cells allows identification of drug targets alternative to MEK and PI3K inhibition.
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Synchronous effects of targeted mitochondrial complex I inhibitors on tumor and immune cells abrogate melanoma progression.
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Synergistic Effects between mTOR Complex 1/2 and Glycolysis Inhibitors in Non-Small-Cell Lung Carcinoma Cells.
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Synergistic effects of co-expression plasmid?based ADAM10-specific siRNA and GRIM-19 on hepatocellular carcinoma in vitro and in vivo.
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Synergistic inhibition of tumor cell proliferation by metformin and mito-metformin in the presence of iron chelators.
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Syntheses and anti-cancer activity of CO-releasing molecules with targeting galactose receptors.
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Synthesis and antitumor activities of transition metal complexes of a bis-Schiff base of 2-hydroxy-1-naphthalenecarboxaldehyde.
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Synthesis and antitumor mechanism of a new iron(iii) complex with 5,7-dichloro-2-methyl-8-quinolinol as ligands.
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Synthesis and biological evaluation of novel platinum complexes of imidazolyl-containing bisphosphonates as potential anticancer agents.
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Synthesis and characterization of a metal complex containing naringin and Cu, and its antioxidant, antimicrobial, antiinflammatory and tumor cell cytotoxicity.
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Synthesis and characterization of a novel Pd(II) complex with the condensation product of 2-(diphenylphosphino)benzaldehyde and ethyl hydrazinoacetate. Cytotoxic activity of the synthesized complex and related Pd(II) and Pt(II) complexes.
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Synthesis and characterization of copper(II) and zinc(II)-based potential chemotherapeutic compounds: their biological evaluation viz. DNA binding profile, cleavage and antimicrobial activity.
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Synthesis and characterization of Cu(II)-based anticancer chemotherapeutic agent targeting topoisomerase I?: in vitro DNA binding, pBR322 cleavage, molecular docking studies and cytotoxicity against human cancer cell lines.
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Synthesis and crystal structure determination of copper(II)-complex: In vitro DNA and HSA binding, pBR322 plasmid cleavage, cell imaging and cytotoxic studies.
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Synthesis and evaluation of iridium(III) complexes on antineoplastic activity against human gastric carcinoma SGC-7901 cells.
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Synthesis and mass spectroscopy kinetics of a novel ternary copper(II) complex with cytotoxic activity against cancer cells.
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Synthesis and selective tumor targeting properties of water soluble porphyrin-Pt(II) conjugates.
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Synthesis and structure of dicopper(II) complexes bridged by N-(5-chloro-2-hydroxyphenyl)-N'-[3-(methy lamino)propyl]oxamide: evaluation of DNA/protein binding, DNA cleavage, and in vitro anticancer activity.
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Synthesis of a DNA-targeting nickel (II) complex with testosterone thiosemicarbazone which exhibits selective cytotoxicity towards human prostate cancer cells (LNCaP).
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Synthesis of a novel platinum(II) complex with 6,7-dichloro-5,8-quinolinedione and the study of its antitumor mechanism in testicular seminoma.
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Synthesis of Cyanoenone-Modified Diterpenoid Analogs as Novel Bmi-1-Mediated Antitumor Agents.
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Synthesis, characterization and in vitro DNA binding and cleavage studies of Cu(II)/Zn(II) dipeptide complexes.
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Synthesis, Characterization, Cellular Uptake, Apoptosis, Cytotoxicity, Dna-Binding, and Antioxidant Activity Studies of Ruthenium(II) Complexes.
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Synthesis, Characterization, DNA/HSA Interactions, and Anticancer Activity of Two Novel Copper(II) Complexes with 4-Chloro-3-Nitrobenzoic Acid Ligand.
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Synthesis, characterization, plasmid cleavage and cytotoxicity of cancer cells by a copper(ii) complex of anthracenyl-terpyridine.
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Synthesis, crystal structure and spectroscopy of bioactive Cd(II) polymeric complex of the non-steroidal anti-inflammatory drug diclofenac sodium: antiproliferative and biological activity.
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Synthesis, crystal structure, cytotoxic activity and DNA-binding properties of the copper (II) and zinc (II) complexes with 1-[3-(2-pyridyl)pyrazol-1-ylmethyl]naphthalene.
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Synthesis, crystal structure, cytotoxicity and action mechanism of a Rh(iii) complex with 8-hydroxy-2-methylquinoline as a ligand.
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Synthesis, DNA binding, cellular DNA lesion and cytotoxicity of a series of new benzimidazole-based Schiff base copper(II) complexes.
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Synthesis, properties, antitumor and antibacterial activity of new Pt(II) and Pd(II) complexes with 2,2'-dithiobis(benzothiazole) ligand.
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Synthesis, structural characterization and biological studies of the triphenyltin(IV) complex with 2-thiobarbituric acid.
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Synthesis, structural characterization, molecular docking study, biological activity of carbon monoxide release molecules as potent antitumor agents.
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Synthesis, Structure Characterization and Antitumor Activity Study of a New Iron(III) Complex of 5-Nitro-8-hydroxylquinoline (HNOQ).
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TAK1-dependent autophagy: A suppressor of fatty liver disease and hepatic oncogenesis.
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Target Discovery of Selective Non-Small-Cell Lung Cancer Toxins Reveals Inhibitors of Mitochondrial Complex I.
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Targeted Approaches toward Understanding and Treating Pulmonary Lymphangioleiomyomatosis (LAM).
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Targeted overexpression of mitochondrial catalase protects against cancer chemotherapy-induced skeletal muscle dysfunction.
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Targeted therapy for mTORC1-driven tumours through HDAC inhibition by exploiting innate vulnerability of mTORC1 hyper-activation.
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Targeting metabolic plasticity in breast cancer cells via mitochondrial complex I modulation.
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Targeting metabolism and AMP-activated kinase with metformin to sensitize non-small cell lung cancer (NSCLC) to cytotoxic therapy: translational biology and rationale for current clinical trials.
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Targeting Mitochondrial Complex I Overcomes Chemoresistance in High OXPHOS Pancreatic Cancer.
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Targeting mitochondrial complex I using BAY 87-2243 reduces melanoma tumor growth.
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Targeting NAD+/PARP DNA Repair Pathway as a Novel Therapeutic Approach to SDHB-Mutated Cluster I Pheochromocytoma and Paraganglioma.
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Targeting of mTORC2 prevents cell migration and promotes apoptosis in breast cancer.
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Targeting Oxidative Phosphorylation Reverses Drug Resistance in Cancer Cells by Blocking Autophagy Recycling.
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Targeting the mitochondrial trifunctional protein restrains tumor growth in oxidative lung carcinomas.
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Targeting the phosphatidylinositol 3-kinase pathway in airway smooth muscle: rationale and promise.
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The BMI1 inhibitor PTC-209 is a potential compound to halt cellular growth in biliary tract cancer cells.
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The cell death regulator GRIM-19 is an inhibitor of signal transducer and activator of transcription 3.
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The copper(II) complexes of new anthrahydrazone ligands: In vitro and in vivo antitumor activity and structure-activity relationship.
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The disubstituted adamantyl derivative LW1564 inhibits the growth of cancer cells by targeting mitochondrial respiration and reducing hypoxia-inducible factor (HIF)-1? accumulation.
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The dual mTOR kinase inhibitor TAK228 inhibits tumorigenicity and enhances radiosensitization in diffuse intrinsic pontine glioma.
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The dual mTORC1 and mTORC2 inhibitor PP242 shows strong antitumor activity in a pheochromocytoma PC12 cell tumor model.
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The E3 Ligase RING1 Targets p53 for Degradation and Promotes Cancer Cell Proliferation and Survival.
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The effect of a gene associated with retinoid-interferon-induced mortality 19 (GRIM-19) on STAT3-induced gene expression in renal carcinoma.
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The expression of mitochondrial complexes I and III correlates with prognosis or diagnosis in retinoblastoma: immunohistochemical and histopathological parameters.
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The functions of Atg8-family proteins in autophagy and cancer: linked or unrelated?
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The genetic and metabolic signature of oncocytic transformation implicates HIF1alpha destabilization.
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The Harmonious Interplay of Amino Acid and Monocarboxylate Transporters Induces the Robustness of Cancer Cells.
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The HMGB1/RAGE inflammatory pathway promotes pancreatic tumor growth by regulating mitochondrial bioenergetics.
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The IFN-beta and retinoic acid-induced cell death regulator GRIM-19 is upregulated during focal cerebral ischemia.
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The interaction of Hemin and Sestrin2 modulates oxidative stress and colon tumor growth.
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The mammalian target of rapamycin complex 1 (mTORC1) in breast cancer: the impact of oestrogen receptor and HER2 pathways.
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The medial and lateral substantia nigra in Parkinson's disease: mRNA profiles associated with higher brain tissue vulnerability.
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The molecular selectivity of UNC3866 inhibitor for Polycomb CBX7 protein from molecular dynamics simulation.
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The mTOR-S6K pathway links growth signalling to DNA damage response by targeting RNF168.
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The mTORC1/S6K1 pathway regulates glutamine metabolism through the eIF4B-dependent control of c-Myc translation.
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The opposite prognostic effect of NDUFS1 and NDUFS8 in lung cancer reflects the oncojanus role of mitochondrial complex I.
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The pleiotropic regulation of cyclin D1 by newly identified sesaminol-binding protein ANT2.
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The Polycomb Orthologues in Teleost Fishes and Their Expression in the Zebrafish Model.
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The Potential of Isoprenoids in Adjuvant Cancer Therapy to Reduce Adverse Effects of Statins.
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The prognostic significance of BMI1 expression in invasive breast cancer is dependent on its molecular subtypes.
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The Splicing Factor SF2 Is Critical for Hyperproliferation and Survival in a TORC1-Dependent Model of Early Tumorigenesis in Drosophila.
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The syntheses and characterizations of vanadium complexes with 1,2-dihydroxyanthraquinone and the structure-effect relationship in their in vitro anticancer activities.
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The TSC1 and TSC2 tumor suppressors are required for proper ER stress response and protect cells from ER stress-induced apoptosis.
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Theranostic Lysosomal Targeting Anticancer and Antimetastatic Agents: Half-Sandwich Iridium(III) Rhodamine Complexes.
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Therapeutic effect of tamoxifen and energy-modulating vitamins on carbohydrate-metabolizing enzymes in breast cancer.
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Thoc1 inhibits cell growth via induction of cell cycle arrest and apoptosis in lung cancer cells.
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TNFAIP8L2/TIPE2 impairs autolysosome reformation via modulating the RAC1-MTORC1 axis.
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TP53INP2 contributes to autophagosome formation by promoting LC3-ATG7 interaction.
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TPGS/Phospholipids Mixed Micelles for Delivery of Icariside II to Multidrug-Resistant Breast Cancer.
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Transglutaminase 2 mediates hypoxia-induced selective mRNA translation via polyamination of 4EBPs.
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Translational studies within the TAMRAD randomized GINECO trial: evidence for mTORC1 activation marker as a predictive factor for everolimus efficacy in advanced breast cancer.
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Translationally controlled tumour protein is associated with podocyte hypertrophy in a mouse model of type 1 diabetes.
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Treatment of Pancreatic Cancer Patient-Derived Xenograft Panel with Metabolic Inhibitors Reveals Efficacy of Phenformin.
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TSC-insensitive Rheb mutations induce oncogenic transformation through a combination of constitutively active mTORC1 signalling and proteome remodelling.
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TSC1 controls IL-1? expression in macrophages via mTORC1-dependent C/EBP? pathway.
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TSC1 Promotes B Cell Maturation but Is Dispensable for Germinal Center Formation.
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Tuberous sclerosis complex 1 regulates dE2F1 expression during development and cooperates with RBF1 to control proliferation and survival.
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Tuberous Sclerosis Complex 1: An Epithelial Tumor Suppressor Essential to Prevent Spontaneous Prostate Cancer in Aged Mice.
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Tumor microenvironment confers mTOR inhibitor resistance in invasive intestinal adenocarcinoma.
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Tumor Microenvironment Modulates Immunological Outcomes of Myeloid Cells with mTORC1 Disruption.
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Tumor necrosis factor-? impairs oligodendroglial differentiation through a mitochondria-dependent process.
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Tumor necrosis factor-alpha (TNF-?)-mediated in vitro human retinal pigment epithelial (RPE) cell migration mainly requires Akt/mTOR complex 1 (mTORC1), but not mTOR complex 2 (mTORC2) signaling.
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Tumor susceptibility gene 101 is required for the maintenance of uterine epithelial cells during embryo implantation.
Neoplasms
Tumor-derived Mutations in the Gene Associated with Retinoid Interferon-induced Mortality (GRIM-19) Disrupt Its Anti-signal Transducer and Activator of Transcription 3 (STAT3) Activity and Promote Oncogenesis.
Neoplasms
Tumorigenesis in tuberous sclerosis complex is autophagy and p62/sequestosome 1 (SQSTM1)-dependent.
Neoplasms
Two molecular pathways initiate mitochondria-dependent dopaminergic neurodegeneration in experimental Parkinson's disease.
Neoplasms
Two new lanthanide complexes with 5-(Pyrazol-1-yl)nicotinic acid: Structures and their anti-cancer properties.
Neoplasms
Tyrosine phosphorylation of lactate dehydrogenase A is important for NADH/NAD(+) redox homeostasis in cancer cells.
Neoplasms
Ubiquilin-mediated Small Molecule Inhibition of Mammalian Target of Rapamycin Complex 1 (mTORC1) Signaling.
Neoplasms
Understanding the complex-I-ty of metformin action: limiting mitochondrial respiration to improve cancer therapy.
Neoplasms
Understanding the interaction of an antitumoral platinum(II) 7-azaindolate complex with proteins and DNA.
Neoplasms
Unravelling the connection between metabolism and tumorigenesis through studies of the liver kinase B1 tumour suppressor.
Neoplasms
Upregulation of 6-phosphofructo-2-kinase (PFKFB3) by hyperactivated mammalian target of rapamycin complex 1 is critical for tumor growth in tuberous sclerosis complex.
Neoplasms
Upregulation of GRIM-19 suppresses the growth of oral squamous cell carcinoma in vitro and in vivo.
Neoplasms
Upregulation of the GRIM-19 gene suppresses invasion and metastasis of human gastric cancer SGC-7901 cell line.
Neoplasms
Validation of a commercially available anti-REDD1 antibody using RNA interference and REDD1-/- mouse embryonic fibroblasts.
Neoplasms
Vps34-mediated macropinocytosis in Tuberous Sclerosis Complex 2-deficient cells supports tumorigenesis.
Neoplasms
Xanthohumol induces generation of reactive oxygen species and triggers apoptosis through inhibition of mitochondrial electron transfer chain complex I.
Neoplasms
[Brucella abortus mutant strain S2308?rfbE promotes maturation and cytokine release of mouse bone marrow-derived dendritic cells].
Neoplasms
[Correlations of GRIM-19 and its target gene product STAT3 to malignancy of human colorectal carcinoma]
Neoplasms
[Identification of multidrug resistance related genes in leukemia by suppression subtractive hybridization]
Neoplasms
[Influence of local anesthetics and narcotics on the energy metabolism of Ehrlich ascites tumor cells.]
Neoplasms, Germ Cell and Embryonal
Synthesis of a novel platinum(II) complex with 6,7-dichloro-5,8-quinolinedione and the study of its antitumor mechanism in testicular seminoma.
Nephritis
Induction monotherapy with sirolimus has selected beneficial effects on glomerular and tubulointersititial injury in nephrotoxic serum nephritis.
Nephrosis
GIV/Girdin Links Vascular Endothelial Growth Factor Signaling to Akt Survival Signaling in Podocytes Independent of Nephrin.
Nephrosis
Telmisartan improves kidney function through inhibition of the oxidative phosphorylation pathway in diabetic rats.
Nephrotic Syndrome
Mitochondrial Complex I Deficiency among Egyptian Pediatric Patients with Steroid-Resistant Nephrotic Syndrome.
Nervous System Diseases
Activating the translational repressor 4E-BP or reducing S6K-GSK3? activity prevents accelerated axon growth induced by hyperactive mTOR in vivo.
Nervous System Diseases
Dysfunction of mitochondrial respiratory chain complex I in neurological disorders: genetics and pathogenetic mechanisms.
Nervous System Diseases
Electrophysiology and pharmacology of striatal neuronal dysfunction induced by mitochondrial complex I inhibition.
Nervous System Diseases
Intranasal Administration of Rotenone Reduces GABAergic Inhibition in the Mouse Insular Cortex Leading to Impairment of LTD and Conditioned Taste Aversion Memory.
Nervous System Diseases
Leukodystrophy associated with mitochondrial complex I deficiency due to a novel mutation in the NDUFAF1 gene.
Nervous System Diseases
Metabolic consequences of NDUFS4 gene deletion in immortalized mouse embryonic fibroblasts.
Nervous System Diseases
Mitochondrial complex I mutations in Caenorhabditis elegans produce cytochrome c oxidase deficiency, oxidative stress and vitamin-responsive lactic acidosis.
Nervous System Diseases
mTORC1 targets the translational repressor 4E-BP2, but not S6 kinase 1/2, to regulate neural stem cell self-renewal in vivo.
Nervous System Diseases
Pathogenetic mechanisms in hereditary dysfunctions of complex I of the respiratory chain in neurological diseases.
Nervous System Diseases
Severe infantile leigh syndrome associated with a rare mitochondrial ND6 mutation, m.14487T>C.
Nervous System Diseases
Succinate and the shortcut to the cure of metformin-induced lactic acidosis.
Nervous System Diseases
The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification.
Neuralgia
Influence of Phosphatidylinositol-3-Kinase/Protein Kinase B-Mammalian Target of Rapamycin Signaling Pathway on the Neuropathic Pain Complicated by Nucleoside Reverse Transcriptase Inhibitors for the Treatment of HIV Infection.
Neuralgia
Inhibition of the mammalian target of rapamycin complex 1 signaling pathway reduces itch behaviour in mice.
Neuralgia
Nociceptor Translational Profiling Reveals the Ragulator-Rag GTPase Complex as a Critical Generator of Neuropathic Pain.
Neurilemmoma
Alterations of oxidative phosphorylation in meningiomas and peripheral nerve sheath tumors.
Neurilemmoma
NF2/merlin is a novel negative regulator of mTOR complex 1, and activation of mTORC1 is associated with meningioma and schwannoma growth.
Neuritis
Changes in the sympathetic innervation of the gut in rotenone treated mice as possible early biomarker for Parkinson's disease.
Neuroblastoma
Alterations in the Mitochondrial Proteome of Neuroblastoma Cells in Response to Complex 1 Inhibition.
Neuroblastoma
Antioxidant properties of rare sugar D-allose: Effects on mitochondrial reactive oxygen species production in Neuro2A cells.
Neuroblastoma
Assessment of the direct and indirect effects of MPP+ and dopamine on the human proteasome: implications for Parkinson's disease aetiology.
Neuroblastoma
Catastrophic ATP loss underlies a metabolic combination therapy tailored for MYCN-amplified neuroblastoma.
Neuroblastoma
Cell death induced by mitochondrial complex I inhibition is mediated by Iron Regulatory Protein 1.
Neuroblastoma
Characterization and time course of MPP+ -induced apoptosis in human SH-SY5Y neuroblastoma cells.
Neuroblastoma
Chronic reduction in complex I function alters calcium signaling in SH-SY5Y neuroblastoma cells.
Neuroblastoma
Chronic, low-dose rotenone reproduces Lewy neurites found in early stages of Parkinson's disease, reduces mitochondrial movement and slowly kills differentiated SH-SY5Y neural cells.
Neuroblastoma
Cytotoxicity of 17 tetrahydroisoquinoline derivatives in SH-SY5Y human neuroblastoma cells is related to mitochondrial NADH-ubiquinone oxidoreductase inhibition.
Neuroblastoma
Decreased expression of the NADH:ubiquinone oxidoreductase (complex I) subunit 4 in 1-methyl-4-phenylpyridinium -treated human neuroblastoma SH-SY5Y cells.
Neuroblastoma
Delta-9-tetrahydrocannabinol protects against MPP+ toxicity in SH-SY5Y cells by restoring proteins involved in mitochondrial biogenesis.
Neuroblastoma
DEPDC5 and NPRL3 modulate cell size, filopodial outgrowth, and localization of mTOR in neural progenitor cells and neurons.
Neuroblastoma
Dependence on electron transport chain function and intracellular signaling of genomic responses in SH-SY5Y cells to the mitochondrial neurotoxin MPP(+).
Neuroblastoma
Detection and determination of reticuline and N-methylcoculaurine in the Annonaceae family using liquid chromatography-tandem mass spectrometry.
Neuroblastoma
Downregulation of PMCA2 increases the vulnerability of midbrain neurons to mitochondrial complex I inhibition.
Neuroblastoma
Fumonisin B(1) inhibits mitochondrial respiration and deregulates calcium homeostasis-Implication to mechanism of cell toxicity.
Neuroblastoma
Glutathione-mediated effects of lithium in decreasing protein oxidation induced by mitochondrial complex I dysfunction.
Neuroblastoma
High doses of nicotinamide prevent oxidative mitochondrial dysfunction in a cellular model and improve motor deficit in a Drosophila model of Parkinson's disease.
Neuroblastoma
Human gammadelta T lymphocytes exert natural and IL-2-induced cytotoxicity to neuroblastoma cells.
Neuroblastoma
Impact of fumonisin B1 on glutamate toxicity and low magnesium-induced seizure activity in neuronal primary culture.
Neuroblastoma
Interaction among mitochondria, mitogen-activated protein kinases, and nuclear factor-kappaB in cellular models of Parkinson's disease.
Neuroblastoma
Mechanism of toxicity of pesticides acting at complex I: relevance to environmental etiologies of Parkinson's disease.
Neuroblastoma
Ndfip1 Prevents Rotenone-Induced Neurotoxicity and Upregulation of ?-Synuclein in SH-SY5Y Cells.
Neuroblastoma
Neuroprotective effects of nicotinamide N-methyltransferase and its metabolite 1-methylnicotinamide.
Neuroblastoma
Nicotinamide N-methyltransferase expression in SH-SY5Y human neuroblastoma cells decreases oxidative stress.
Neuroblastoma
Nicotinamide N-methyltransferase increases complex I activity in SH-SY5Y cells via sirtuin 3.
Neuroblastoma
Novel imine antioxidants at low nanomolar concentrations protect dopaminergic cells from oxidative neurotoxicity.
Neuroblastoma
Novel incretin analogues improve autophagy and protect from mitochondrial stress induced by rotenone in SH-SY5Y cells.
Neuroblastoma
Rotenone down-regulates HSPA8/hsc70 chaperone protein in vitro: A new possible toxic mechanism contributing to Parkinson's disease.
Neuroblastoma
Rotenone Upregulates Alpha-Synuclein and Myocyte Enhancer Factor 2D Independently from Lysosomal Degradation Inhibition.
Neuroblastoma
Sequential and concerted gene expression changes in a chronic in vitro model of parkinsonism.
Neuroblastoma
Sorafenib-induced mitochondrial complex I inactivation and cell death in human neuroblastoma cells.
Neuroblastoma
Sp1 expression is disrupted in schizophrenia; a possible mechanism for the abnormal expression of mitochondrial complex I genes, NDUFV1 and NDUFV2.
Neuroblastoma
TCF11/Nrf1-mediated induction of proteasome expression prevents cytotoxicity by Rotenone.
Neuroblastoma
The dopamine metabolite aminochrome inhibits mitochondrial complex I and modifies the expression of iron transporters DMT1 and FPN1.
Neuroblastoma
The expression of nicotinamide N-methyltransferas increases ATP synthesis and protects SH-SY5Y neuroblastoma cells against the toxicity of complex I inhibitors.
Neuroblastoma
The IFN-beta and retinoic acid-induced cell death regulator GRIM-19 is upregulated during focal cerebral ischemia.
Neuroblastoma
The link between mitochondrial complex I and brain-derived neurotrophic factor in SH-SY5Y cells--The potential of JNX1001 as a therapeutic agent.
Neuroblastoma
The mechanism of alternative splicing of the X-linked NDUFB11 gene of the respiratory chain complex I, impact of rotenone treatment in neuroblastoma cells.
Neuroblastoma
The plasma membrane redox enzyme NQO1 sustains cellular energetics and protects human neuroblastoma cells against metabolic and proteotoxic stress.
Neuroblastoma
The single subunit NADH dehydrogenase reduces generation of reactive oxygen species from complex I.
Neurocutaneous Syndromes
The coding and non-coding transcriptional landscape of subependymal giant cell astrocytomas.
Neurodegenerative Diseases
A novel NDUFA1 mutation leads to a progressive mitochondrial complex I-specific neurodegenerative disease.
Neurodegenerative Diseases
A single-subunit NADH-quinone oxidoreductase renders resistance to mammalian nerve cells against complex I inhibition.
Neurodegenerative Diseases
A small molecule inhibitor of Rheb selectively targets mTORC1 signaling.
Neurodegenerative Diseases
Accessory Subunits of the Matrix Arm of Mitochondrial Complex I with a Focus on Subunit NDUFS4 and Its Role in Complex I Function and Assembly.
Neurodegenerative Diseases
Amyloid Precursor Protein Mediates Neuronal Protection from Rotenone Toxicity.
Neurodegenerative Diseases
Analysis of the mitochondrial genome of cheetahs (Acinonyx jubatus) with neurodegenerative disease.
Neurodegenerative Diseases
Anatomic and disease specificity of NADH CoQ1 reductase (complex I) deficiency in Parkinson's disease.
Neurodegenerative Diseases
Application of the obligate aerobic yeast Yarrowia lipolytica as a eucaryotic model to analyse Leigh syndrome mutations in the complex I core subunits PSST and TYKY.
Neurodegenerative Diseases
Bi-allelic pathogenic variants in NDUFC2 cause early-onset Leigh syndrome and stalled biogenesis of complex I.
Neurodegenerative Diseases
Biochemical consequences of two clinically relevant ND-gene mutations in Escherichia coli respiratory complex I.
Neurodegenerative Diseases
Bioenergetics and the formation of mitochondrial reactive oxygen species.
Neurodegenerative Diseases
Brain mitochondrial dysfunction in aging, neurodegeneration, and Parkinson's disease.
Neurodegenerative Diseases
Brain, skeletal muscle and platelet homogenate mitochondrial function in Parkinson's disease.
Neurodegenerative Diseases
C9orf72 ALS-FTD: recent evidence for dysregulation of the autophagy-lysosome pathway at multiple levels.
Neurodegenerative Diseases
Ca2+-induced oxidative stress in brain mitochondria treated with the respiratory chain inhibitor rotenone.
Neurodegenerative Diseases
Coenzyme Q cytoprotective mechanisms for mitochondrial complex I cytopathies involves NAD(P)H: quinone oxidoreductase 1(NQO1).
Neurodegenerative Diseases
Complex I deficiency primes Bax-dependent neuronal apoptosis through mitochondrial oxidative damage.
Neurodegenerative Diseases
Complex I is rate-limiting for oxygen consumption in the nerve terminal.
Neurodegenerative Diseases
Complex I specific increase in superoxide formation and respiration rate by PrP-null mouse brain mitochondria.
Neurodegenerative Diseases
Driving neural regeneration through the mammalian target of rapamycin.
Neurodegenerative Diseases
Dual loss of succinate dehydrogenase (SDH) and complex I activity is necessary to recapitulate the metabolic phenotype of SDH mutant tumors.
Neurodegenerative Diseases
Dynamics of Human Mitochondrial Complex I Assembly: Implications for Neurodegenerative Diseases.
Neurodegenerative Diseases
Effect of monovalent cations on the kinetics of hypoxic conformational change of mitochondrial complex I.
Neurodegenerative Diseases
Electrophysiology and pharmacology of striatal neuronal dysfunction induced by mitochondrial complex I inhibition.
Neurodegenerative Diseases
Essential regions in the membrane domain of bacterial complex I (NDH-1): the machinery for proton translocation.
Neurodegenerative Diseases
Five decades of research on mitochondrial NADH-quinone oxidoreductase (complex I).
Neurodegenerative Diseases
Functional expression of the single subunit NADH dehydrogenase in mitochondria in vivo: a potential therapy for complex I deficiencies.
Neurodegenerative Diseases
Glutaredoxin is essential for maintenance of brain mitochondrial complex I: studies with MPTP.
Neurodegenerative Diseases
Idebenone Has Distinct Effects on Mitochondrial Respiration in Cortical Astrocytes Compared to Cortical Neurons Due to Differential NQO1 Activity.
Neurodegenerative Diseases
Immunocytochemical characterization of the mitochondrially encoded ND1 subunit of complex I (NADH : ubiquinone oxidoreductase) in rat brain.
Neurodegenerative Diseases
In vivo complementation of complex I by the yeast Ndi1 enzyme. Possible application for treatment of Parkinson disease.
Neurodegenerative Diseases
Inhibition of mitochondrial complex II in neuronal cells triggers unique pathways culminating in autophagy with implications for neurodegeneration.
Neurodegenerative Diseases
Inhibition of the glutamine transporter SNAT1 confers neuroprotection in mice by modulating the mTOR-autophagy system.
Neurodegenerative Diseases
Intrastriatal neurotoxin injections reduce in vitro and in vivo binding of radiolabeled rotenoids to mitochondrial complex I.
Neurodegenerative Diseases
Mechanism of cell death caused by complex I defects in a rat dopaminergic cell line.
Neurodegenerative Diseases
Melatonin protects against 6-OHDA-induced neurotoxicity in rats: a role for mitochondrial complex I activity.
Neurodegenerative Diseases
Mesencephalic complex I deficiency does not correlate with parkinsonism in mitochondrial DNA maintenance disorders.
Neurodegenerative Diseases
Metallothionein 1 Overexpression Does Not Protect Against Mitochondrial Disease Pathology in Ndufs4 Knockout Mice.
Neurodegenerative Diseases
Metformin delays neurological symptom onset in a mouse model of neuronal complex I deficiency.
Neurodegenerative Diseases
Mitochondria in the nervous system: From Health to Disease, Part I.
Neurodegenerative Diseases
Mitochondrial Complex 1 Activity Measured by Spectrophotometry Is Reduced across All Brain Regions in Ageing and More Specifically in Neurodegeneration.
Neurodegenerative Diseases
Mitochondrial complex I deficiency leads to the retardation of early embryonic development in Ndufs4 knockout mice.
Neurodegenerative Diseases
Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease.
Neurodegenerative Diseases
Mitochondrial network complexity and pathological decrease in complex I activity are tightly correlated in isolated human complex I deficiency.
Neurodegenerative Diseases
Multivariate meta-analyses of mitochondrial complex I and IV in major depressive disorder, bipolar disorder, schizophrenia, Alzheimer disease, and Parkinson disease.
Neurodegenerative Diseases
Neuronal MHC-I expression and its implications in synaptic function, axonal regeneration and Parkinson's and other brain diseases.
Neurodegenerative Diseases
Oxidation of multiple MiT/TFE transcription factors links oxidative stress to transcriptional control of autophagy and lysosome biogenesis.
Neurodegenerative Diseases
Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage.
Neurodegenerative Diseases
Partial inhibition of complex I activity increases Ca-independent glutamate release rates from depolarized synaptosomes.
Neurodegenerative Diseases
Psychotropic and neurological medication effects on mitochondrial complex I and IV in rodent models.
Neurodegenerative Diseases
Regulation of brain mitochondrial H2O2 production by membrane potential and NAD(P)H redox state.
Neurodegenerative Diseases
Restoration of mitochondrial function in cells with complex I deficiency.
Neurodegenerative Diseases
Rotenone induces oxidative stress and dopaminergic neuron damage in organotypic substantia nigra cultures.
Neurodegenerative Diseases
Structure/Function Relations in AIFM1 Variants Associated with Neurodegenerative Disorders.
Neurodegenerative Diseases
Submitochondrial fragments of brain mitochondria: general characteristics and catalytic properties of NADH:ubiquinone oxidoreductase (complex I).
Neurodegenerative Diseases
The antioxidant Trolox restores mitochondrial membrane potential and Ca2+ -stimulated ATP production in human complex I deficiency.
Neurodegenerative Diseases
The mechanism of proton translocation in respiratory complex I from molecular dynamics.
Neurodegenerative Diseases
The reduction of NADH ubiquinone oxidoreductase 24- and 75-kDa subunits in brains of patients with Down syndrome and Alzheimer's disease.
Neurodegenerative Diseases
Titrating the effects of mitochondrial complex I impairment in the cell physiology.
Neurodegenerative Diseases
Transcriptome analysis of a rotenone model of parkinsonism reveals complex I-tied and -untied toxicity mechanisms common to neurodegenerative diseases.
Neurodegenerative Diseases
Treatment with AAV1-Rheb(S16H) provides neuroprotection in a mouse model of photothrombosis-induced ischemic stroke.
Neurodegenerative Diseases
Two molecular pathways initiate mitochondria-dependent dopaminergic neurodegeneration in experimental Parkinson's disease.
Neuroendocrine Tumors
A phase 2 study of an oral mTORC1/mTORC2 kinase inhibitor (CC-223) for non-pancreatic neuroendocrine tumors with or without carcinoid symptoms.
Neuroendocrine Tumors
Pancreatic Neuroendocrine Tumor in a Young Child With Tuberous Sclerosis Complex 1.
Neuroendocrine Tumors
Phase II Study of BEZ235 versus Everolimus in Patients with Mammalian Target of Rapamycin Inhibitor-Naïve Advanced Pancreatic Neuroendocrine Tumors.
Neurofibroma
Alterations of oxidative phosphorylation in meningiomas and peripheral nerve sheath tumors.
Neurofibromatoses
Neurofibromatosis Type 1: A Novel NF1 Mutation Associated with Mitochondrial Complex I Deficiency.
Neurofibromatosis 1
Dysregulation of Mammalian Target of Rapamycin Signaling in Mouse Models of Autism.
Neurofibromatosis 1
Neurofibromatosis Type 1: A Novel NF1 Mutation Associated with Mitochondrial Complex I Deficiency.
Neurofibrosarcoma
Effective in vivo targeting of the mammalian target of rapamycin pathway in malignant peripheral nerve sheath tumors.
Neuroinflammatory Diseases
An adverse outcome pathway for parkinsonian motor deficits associated with mitochondrial complex I inhibition.
Neuroinflammatory Diseases
Comparing amyloid-? deposition, neuroinflammation, glucose metabolism, and mitochondrial complex I activity in brain: a PET study in aged monkeys.
Neuroinflammatory Diseases
Low-Grade Inflammation Aggravates Rotenone Neurotoxicity and Disrupts Circadian Clock Gene Expression in Rats.
Neuroinflammatory Diseases
Sodium orthovanadate improves learning and memory in intracerebroventricular-streptozotocin rat model of Alzheimer's disease through modulation of brain insulin resistance induced tau pathology.
Neurologic Manifestations
Early-onset cataracts, spastic paraparesis, and ataxia caused by a novel mitochondrial tRNAGlu (MT-TE) gene mutation causing severe complex I deficiency: a clinical, molecular, and neuropathologic study.
Neurologic Manifestations
Progressive myoclonic epilepsy as an adult-onset manifestation of Leigh syndrome due to m.14487T>C.
Neuroma, Acoustic
Mechanism-based modeling of the clinical effects of bevacizumab and everolimus on vestibular schwannomas of patients with neurofibromatosis type 2.
Neuromuscular Diseases
Production of Reactive Oxygen Species by Complex I (NADH:Ubiquinone Oxidoreductase) from Escherichia coli and Comparison to the Enzyme from Mitochondria.
Neuromuscular Diseases
The respiratory complexes I from the mitochondria of two Pichia species.
Neuronal Ceroid-Lipofuscinoses
Btn3 is a negative regulator of Btn2-mediated endosomal protein trafficking and prion curing in yeast.
Neuronal Ceroid-Lipofuscinoses
Quantitative analysis of proteins of metabolism by reverse phase protein microarrays identifies potential biomarkers of rare neuromuscular diseases.
Non-alcoholic Fatty Liver Disease
A review of the studies on food-derived factors which regulate energy metabolism via the modulation of lipid-sensing nuclear receptors.
Non-alcoholic Fatty Liver Disease
Activation of mTORC1 disrupted LDL receptor pathway: A potential new mechanism for the progression of non-alcoholic fatty liver disease.
Non-alcoholic Fatty Liver Disease
Epigenetic modification of liver mitochondrial DNA is associated with histological severity of nonalcoholic fatty liver disease.
Non-alcoholic Fatty Liver Disease
Fructose leads to hepatic steatosis in zebrafish that is reversed by mechanistic target of rapamycin (mTOR) inhibition.
Non-alcoholic Fatty Liver Disease
Highly expressed MT-ND3 positively associated with histological severity of hepatic steatosis.
Non-alcoholic Fatty Liver Disease
Inhibition of tumor propellant glutathione peroxidase 4 induces ferroptosis in cancer cells and enhances anticancer effect of cisplatin.
Non-alcoholic Fatty Liver Disease
Lipotoxicity-induced STING1 activation stimulates MTORC1 and restricts hepatic lipophagy.
Non-alcoholic Fatty Liver Disease
Mitochondrial complex I subunits are decreased in murine nonalcoholic fatty liver disease: implication of peroxynitrite.
Non-alcoholic Fatty Liver Disease
Mitochondrial dysfunction in rat with nonalcoholic fatty liver Involvement of complex I, reactive oxygen species and cardiolipin.
Non-alcoholic Fatty Liver Disease
SQSTM1/p62 activates NFE2L2/NRF2 via ULK1-mediated autophagic KEAP1 degradation and protects mouse liver from lipotoxicity.
Non-alcoholic Fatty Liver Disease
The Antidiabetic Drug Lobeglitazone Protects Mice From Lipogenesis-Induced Liver Injury via Mechanistic Target of Rapamycin Complex 1 Inhibition.
Non-alcoholic Fatty Liver Disease
The ménage à trois of autophagy, lipid droplets and liver disease.
Non-alcoholic Fatty Liver Disease
TXNIP/VDUP1 attenuates steatohepatitis via autophagy and fatty acid oxidation.
Obesity
A Low-Protein Diet for Diabetic Kidney Disease: Its Effect and Molecular Mechanism, an Approach from Animal Studies.
Obesity
Activation of ER stress and mTORC1 suppresses hepatic sortilin-1 levels in obese mice.
Obesity
Activin signaling mediates muscle-to-adipose communication in a mitochondria dysfunction-associated obesity model.
Obesity
Adenosine Triphosphate Production of Muscle Mitochondria after Acute Exercise in Lean and Obese Humans.
Obesity
Aifm2, a NADH Oxidase, Supports Robust Glycolysis and Is Required for Cold- and Diet-Induced Thermogenesis.
Obesity
Berberine alleviates lipid metabolism disorders via inhibition of mitochondrial complex I in gut and liver.
Obesity
Defective Leptin-AMP-Dependent Kinase Pathway Induces Nitric Oxide Release and Contributes to Mitochondrial Dysfunction and Obesity in ob/ob Mice.
Obesity
Donepezil Prevents Inhibition of Cerebral Energetic Metabolism Without Altering Behavioral Parameters in Animal Model of Obesity.
Obesity
Dysregulation of mitochondrial function and biogenesis modulators in adipose tissue of obese children.
Obesity
Enhanced liver but not muscle OXPHOS in diabetes and reduced glucose output by complex I inhibition.
Obesity
Hepatic mTORC1 Opposes Impaired Insulin Action to Control Mitochondrial Metabolism in Obesity.
Obesity
Mammalian target of rapamycin complex 1 (mTORC1) signaling in energy balance and obesity.
Obesity
Maternal Obesity Increases Oxidative Stress in Placenta and It Is Associated With Intestinal Microbiota.
Obesity
NDUFAB1 protects against obesity and insulin resistance by enhancing mitochondrial metabolism.
Obesity
Pharmacological inhibition of S6K1 increases glucose metabolism and Akt signalling in vitro and in diet-induced obese mice.
Obesity
Placental mTOR complex 1 regulates fetal programming of obesity and insulin resistance in mice.
Obesity
Prepregnancy maternal diabetes combined with obesity impairs placental mitochondrial function involving Nrf2/ARE pathway and detrimentally alters metabolism of offspring.
Obesity
Short-term high-fat diet induces muscle fiber type-selective anabolic resistance to resistance exercise.
Obesity
The role of hypothalamic mammalian target of rapamycin complex 1 signaling in diet-induced obesity.
Obesity
[Mechanism of Electropuncture for Reducing Diet-induced Obesity Rat Weight through Hypothala- mus TSC1 -mTOR Signal Pathway].
Obesity, Morbid
The GID ubiquitin ligase complex is a regulator of AMPK activity and organismal lifespan.
Onchocerciasis
Analysis of genetic variation in ribosomal DNA internal transcribed spacer and the NADH dehydrogenase subunit 4 mitochondrial genes of the onchocerciasis vector Simulium ochraceum.
Ophthalmoplegia
Spontaneous Kearns-Sayre/chronic external ophthalmoplegia plus syndrome associated with a mitochondrial DNA deletion: a slip-replication model and metabolic therapy.
Ophthalmoplegia, Chronic Progressive External
A novel mitochondrial DNA deletion producing progressive external ophthalmoplegia associated with multiple sclerosis.
Ophthalmoplegia, Chronic Progressive External
Chronic progressive external ophthalmoplegia with NADH-CoQ reductase deficiency: report of a case.
Optic Atrophy
Downregulation of apoptosis-inducing factor in Harlequin mice induces progressive and severe optic atrophy which is durably prevented by AAV2-AIF1 gene therapy.
Optic Atrophy
Familial multisystem degeneration with parkinsonism associated with the 11778 mitochondrial DNA mutation.
Optic Atrophy
Iron-induced energy supply deficiency and mitochondrial fragmentation in neurons.
Optic Atrophy
Mutant NADH dehydrogenase subunit 4 gene delivery to mitochondria by targeting sequence-modified adeno-associated virus induces visual loss and optic atrophy in mice.
Optic Atrophy
Neuroglobin Gene Therapy Prevents Optic Atrophy and Preserves Durably Visual Function in Harlequin Mice.
Optic Atrophy
Nuclear expression of mitochondrial ND4 leads to the protein assembling in complex I and prevents optic atrophy and visual loss.
Optic Atrophy
Oestrogens ameliorate mitochondrial dysfunction in Leber's hereditary optic neuropathy.
Optic Atrophy
Optic atrophy-associated TMEM126A is an assembly factor for the ND4-module of mitochondrial complex I.
Optic Atrophy
Photoreceptors in a mouse model of Leigh syndrome are capable of normal light-evoked signaling.
Optic Atrophy
Progressive optic atrophy in a retinal ganglion cell-specific mouse model of complex I deficiency.
Optic Atrophy
Renal cold storage followed by transplantation impairs expression of key mitochondrial fission and fusion proteins.
Optic Atrophy
Targeting Multiple Mitochondrial Processes by a Metabolic Modulator Prevents Sarcopenia and Cognitive Decline in SAMP8 Mice.
Optic Atrophy
The mitochondrial 13513G > A mutation is most frequent in Leigh syndrome combined with reduced complex I activity, optic atrophy and/or Wolff-Parkinson-White.
Optic Atrophy, Autosomal Dominant
Defective Mitochondrial Adenosine Triphosphate Production in Skeletal Muscle From Patients With Dominant Optic Atrophy Due to OPA1 Mutations.
Optic Atrophy, Autosomal Dominant
Divergent skeletal muscle mitochondrial phenotype between male and female patients with chronic heart failure.
Optic Atrophy, Autosomal Dominant
Hereditary optic neuropathies share a common mitochondrial coupling defect.
Optic Atrophy, Autosomal Dominant
Mitochondrial DNA disturbances and deregulated expression of oxidative phosphorylation and mitochondrial fusion proteins in sporadic inclusion body myositis.
Optic Atrophy, Autosomal Dominant
Optic atrophy 1 mediates mitochondria remodeling and dopaminergic neurodegeneration linked to complex I deficiency.
Optic Atrophy, Autosomal Dominant
The idebenone metabolite QS10 restores electron transfer in complex I and coenzyme Q defects.
Optic Atrophy, Hereditary, Leber
A family with 3460G>A and 11778G>A mutations and haplogroup analysis of Polish Leber hereditary optic neuropathy patients.
Optic Atrophy, Hereditary, Leber
A mitochondrial DNA mutation at nucleotide pair 14459 of the NADH dehydrogenase subunit 6 gene associated with maternally inherited Leber hereditary optic neuropathy and dystonia.
Optic Atrophy, Hereditary, Leber
A novel mitochondrial ND5 (MTND5) gene mutation giving isolated exercise intolerance.
Optic Atrophy, Hereditary, Leber
A plasma metabolomic signature of Leber hereditary optic neuropathy showing taurine and nicotinamide deficiencies.
Optic Atrophy, Hereditary, Leber
Allelic mutations of the fourth subunit of NADH dehydrogenase are not pathogenetically important in 11778-negative Leber hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Alternative, simultaneous complex I mitochondrial DNA mutations in Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Analysis of the pathogenic human mitochondrial mutation ND1/3460, and mutations of strictly conserved residues in its vicinity, using the bacterium Paracoccus denitrificans.
Optic Atrophy, Hereditary, Leber
Association of the mtDNA m.4171C>A/MT-ND1 mutation with both optic neuropathy and bilateral brainstem lesions.
Optic Atrophy, Hereditary, Leber
Bioactivity and gene expression profiles of hiPSC-generated retinal ganglion cells in MT-ND4 mutated Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Biochemical features of mtDNA 14484 (ND6/M64V) point mutation associated with Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Bioenergetics of mitochondrial diseases associated with mtDNA mutations.
Optic Atrophy, Hereditary, Leber
Bipolar cell reduction precedes retinal ganglion neuron loss in a complex 1 knockout mouse model.
Optic Atrophy, Hereditary, Leber
Caspase-independent death of Leber's hereditary optic neuropathy cybrids is driven by energetic failure and mediated by AIF and Endonuclease G.
Optic Atrophy, Hereditary, Leber
Catalytic activity of complex I in cell lines that possess replacement mutations in the ND genes in Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Cells bearing mutations causing Leber's hereditary optic neuropathy are sensitized to Fas-Induced apoptosis.
Optic Atrophy, Hereditary, Leber
Changes in mitochondrial complex I activity and coenzyme Q binding site in Leber's hereditary optic neuropathy (LHON).
Optic Atrophy, Hereditary, Leber
Cigarette toxicity triggers Leber's hereditary optic neuropathy by affecting mtDNA copy number, oxidative phosphorylation and ROS detoxification pathways.
Optic Atrophy, Hereditary, Leber
Complex I deficiency primes Bax-dependent neuronal apoptosis through mitochondrial oxidative damage.
Optic Atrophy, Hereditary, Leber
Complex I mutations synergize to worsen the phenotypic expression of Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Construction and detection of a novel type of recombinant human rAAV2/2-ND4.
Optic Atrophy, Hereditary, Leber
Cytochrome b mutations in Leber hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Defective Mitochondrial Adenosine Triphosphate Production in Skeletal Muscle From Patients With Dominant Optic Atrophy Due to OPA1 Mutations.
Optic Atrophy, Hereditary, Leber
Differential production of superoxide by neuronal mitochondria.
Optic Atrophy, Hereditary, Leber
Differentiation-specific effects of LHON mutations introduced into neuronal NT2 cells.
Optic Atrophy, Hereditary, Leber
DNAJC30 biallelic mutations extend mitochondrial complex I-deficient phenotypes to include recessive Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Efficient mitochondrial biogenesis drives incomplete penetrance in Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Exercise Intolerance and Myoglobinuria Associated with a Novel Maternally Inherited MT-ND1 Mutation.
Optic Atrophy, Hereditary, Leber
Fifteen novel mutations in the mitochondrial NADH dehydrogenase subunit 1, 2, 3, 4, 4L, 5 and 6 genes from Iranian patients with Leber's hereditary optic neuropathy (LHON).
Optic Atrophy, Hereditary, Leber
Food derived respiratory complex I inhibitors modify the effect of Leber hereditary optic neuropathy mutations.
Optic Atrophy, Hereditary, Leber
Functional analysis of lymphoblast and cybrid mitochondria containing the 3460, 11778, or 14484 Leber's hereditary optic neuropathy mitochondrial DNA mutation.
Optic Atrophy, Hereditary, Leber
Functional consequences of the 3460-bp mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Gene Therapy for Leber Hereditary Optic Neuropathy: Initial Results.
Optic Atrophy, Hereditary, Leber
Generation of patient-specific induced pluripotent stem cells from Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Genetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystonia.
Optic Atrophy, Hereditary, Leber
Glutamate Stimulation Dysregulates AMPA Receptors-Induced Signal Transduction Pathway in Leber's Inherited Optic Neuropathy Patient-Specific hiPSC-Derived Retinal Ganglion Cells.
Optic Atrophy, Hereditary, Leber
Glutathione depletion in antioxidant defense of differentiated NT2-LHON cybrids.
Optic Atrophy, Hereditary, Leber
Hereditary optic neuropathies share a common mitochondrial coupling defect.
Optic Atrophy, Hereditary, Leber
Homoplasmic and exclusive ND4 gene mutation in Japanese pedigrees with Leber's disease.
Optic Atrophy, Hereditary, Leber
Idebenone Has Distinct Effects on Mitochondrial Respiration in Cortical Astrocytes Compared to Cortical Neurons Due to Differential NQO1 Activity.
Optic Atrophy, Hereditary, Leber
Idebenone protects against retinal damage and loss of vision in a mouse model of Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Identification of small molecules that improve ATP synthesis defects conferred by Leber's hereditary optic neuropathy mutations.
Optic Atrophy, Hereditary, Leber
Impaired complex I repair causes recessive Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
In vivo labeling of mitochondrial complex I (NADH:ubiquinone oxidoreductase) in rat brain using [(3)H]dihydrorotenone.
Optic Atrophy, Hereditary, Leber
Induction of rapid and highly efficient expression of the human ND4 complex I subunit in the mouse visual system by self-complementary adeno-associated virus.
Optic Atrophy, Hereditary, Leber
Inhibition of mitochondrial function induces an integrated stress response in oligodendroglia.
Optic Atrophy, Hereditary, Leber
Intravitreal delivery of AAV-NDI1 provides functional benefit in a murine model of Leber hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Leber hereditary optic neuropathy mutations in the ND6 subunit of mitochondrial complex I affect ubiquinone reduction kinetics in a bacterial model of the enzyme.
Optic Atrophy, Hereditary, Leber
Leber's hereditary optic neuropathy (LHON) pathogenic mutations induce mitochondrial-dependent apoptotic death in transmitochondrial cells incubated with galactose medium.
Optic Atrophy, Hereditary, Leber
Leber's hereditary optic neuropathy (LHON)-associated ND5 12338T>C mutation altered the assembly and function of complex I, apoptosis and mitophagy.
Optic Atrophy, Hereditary, Leber
Leber's hereditary optic neuropathy and complex I deficiency in muscle.
Optic Atrophy, Hereditary, Leber
Leber's hereditary optic neuropathy is multiorgan not mono-organ.
Optic Atrophy, Hereditary, Leber
Leber's hereditary optic neuropathy with dystonia in a Japanese family.
Optic Atrophy, Hereditary, Leber
Leigh disease caused by the mitochondrial DNA G14459A mutation in unrelated families.
Optic Atrophy, Hereditary, Leber
LHON/MELAS overlap mutation in ND1 subunit of mitochondrial complex I affects ubiquinone binding as revealed by modeling in Escherichia coli NDH-1.
Optic Atrophy, Hereditary, Leber
Methylene blue prevents neurodegeneration caused by rotenone in the retina.
Optic Atrophy, Hereditary, Leber
Microphotometric analysis of NADH-tetrazolium reductase deficiency in fibroblasts of patients with Leber hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Mitochondrial complex I deficiency leads to inflammation and retinal ganglion cell death in the Ndufs4 mouse.
Optic Atrophy, Hereditary, Leber
Mitochondrial Complex I: structure, function, and implications in neurodegeneration.
Optic Atrophy, Hereditary, Leber
Mitochondrial DNA background modulates the assembly kinetics of OXPHOS complexes in a cellular model of mitochondrial disease.
Optic Atrophy, Hereditary, Leber
Mitochondrial DNA complex I and III mutations associated with Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Mitochondrial DNA mutations in complex I and tRNA genes in Parkinson's disease.
Optic Atrophy, Hereditary, Leber
Mitochondrial DNA polymorphism in Finnish families with Leber's hereditary optic neuroretinopathy.
Optic Atrophy, Hereditary, Leber
Mitochondrial DNA variant associated with Leber hereditary optic neuropathy and high-altitude Tibetans.
Optic Atrophy, Hereditary, Leber
Mitochondrial transport mediates survival of retinal ganglion cells in affected LHON patients.
Optic Atrophy, Hereditary, Leber
Mitophagy activation repairs Leber's hereditary optic neuropathy-associated mitochondrial dysfunction and improves cell survival.
Optic Atrophy, Hereditary, Leber
Mutant NADH dehydrogenase subunit 4 gene delivery to mitochondria by targeting sequence-modified adeno-associated virus induces visual loss and optic atrophy in mice.
Optic Atrophy, Hereditary, Leber
Mutation in NDUFA13/GRIM19 leads to early onset hypotonia, dyskinesia and sensorial deficiencies, and mitochondrial complex I instability.
Optic Atrophy, Hereditary, Leber
Neurodegeneration produced by rotenone in the mouse retina: a potential model to investigate environmental pesticide contributions to neurodegenerative diseases.
Optic Atrophy, Hereditary, Leber
Neuropathology of white matter disease in Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Next-generation sequencing of mitochondrial targeted AAV transfer of human ND4 in mice.
Optic Atrophy, Hereditary, Leber
Nuclear expression of mitochondrial ND4 leads to the protein assembling in complex I and prevents optic atrophy and visual loss.
Optic Atrophy, Hereditary, Leber
Oestrogens ameliorate mitochondrial dysfunction in Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Optic neuropathy induced by reductions in mitochondrial superoxide dismutase.
Optic Atrophy, Hereditary, Leber
Partial mitochondrial complex I inhibition induces oxidative damage and perturbs glutamate transport in primary retinal cultures. Relevance to Leber Hereditary Optic Neuropathy (LHON).
Optic Atrophy, Hereditary, Leber
Patients with Leber hereditary optic neuropathy fail to compensate impaired oxidative phosphorylation.
Optic Atrophy, Hereditary, Leber
Peculiar combinations of individually non-pathogenic missense mitochondrial DNA variants cause low penetrance Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
Propagation and Selectivity of Axonal Loss in Leber Hereditary Optic Neuropathy.
Optic Atrophy, Hereditary, Leber
Respiratory complex I dysfunction due to mitochondrial DNA mutations shifts the voltage threshold for opening of the permeability transition pore toward resting levels.
Optic Atrophy, Hereditary, Leber
Sequence homology of NADH CoQ reductase subunit IV with nucleotide-requiring enzymes.
Optic Atrophy, Hereditary, Leber
Severe impairment of complex I-driven adenosine triphosphate synthesis in leber hereditary optic neuropathy cybrids.
Optic Atrophy, Hereditary, Leber
SOD2 gene transfer protects against optic neuropathy induced by deficiency of complex I.
Optic Atrophy, Hereditary, Leber
The 14484 ND6 mtDNA mutation in Leber hereditary optic neuropathy does not affect fibroblast complex I activity.
Optic Atrophy, Hereditary, Leber
The background of mitochondrial DNA haplogroup J increases the sensitivity of Leber's hereditary optic neuropathy cells to 2,5-hexanedione toxicity.
Optic Atrophy, Hereditary, Leber
The idebenone metabolite QS10 restores electron transfer in complex I and coenzyme Q defects.
Optic Atrophy, Hereditary, Leber
The m.3890G>A/MT-ND1 mtDNA rare pathogenic variant: Expanding clinical and MRI phenotypes.
Optic Atrophy, Hereditary, Leber
The MELAS mutations 3946 and 3949 perturb the critical structure in a conserved loop of the ND1 subunit of mitochondrial complex I.
Optic Atrophy, Hereditary, Leber
The metabolomic signature of Leber's hereditary optic neuropathy reveals endoplasmic reticulum stress.
Optic Atrophy, Hereditary, Leber
The mitochondrial DNA mutation ND6*14,484C associated with leber hereditary optic neuropathy, leads to deficiency of complex I of the respiratory chain.
Optic Atrophy, Hereditary, Leber
The ND1 gene of complex I is a mutational hot spot for Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
The NDUFB11 gene is not a modifier in Leber hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
The nuoM arg368his mutation in NADH:ubiquinone oxidoreductase from Rhodobacter capsulatus: a model for the human nd4-11778 mtDNA mutation associated with Leber's hereditary optic neuropathy.
Optic Atrophy, Hereditary, Leber
The spectrum of mitochondrial DNA mutations in families with Leber hereditary optic neuroretinopathy.
Optic Atrophy, Hereditary, Leber
Therapeutic applications of the TAT-mediated protein transduction system for complex I deficiency and other mitochondrial diseases.
Optic Atrophy, Hereditary, Leber
Therapeutic strategies for Leber's hereditary optic neuropathy: A current update.
Optic Atrophy, Hereditary, Leber
Use of transmitochondrial cybrids to assign a complex I defect to the mitochondrial DNA-encoded NADH dehydrogenase subunit 6 gene mutation at nucleotide pair 14459 that causes Leber hereditary optic neuropathy and dystonia.
Optic Atrophy, Hereditary, Leber
XIAP Protects Retinal Ganglion Cells in the Mutant ND4 Mouse Model of Leber Hereditary Optic Neuropathy.
Optic Atrophy, Hereditary, Leber
Yeast NDI1 improves oxidative phosphorylation capacity and increases protection against oxidative stress and cell death in cells carrying a Leber's hereditary optic neuropathy mutation.
Optic Atrophy, Hereditary, Leber
[Molecular genetic analysis of Leber's hereditary optic neuropathy with the 3460 mutation in Japanese pedigrees]
Optic Nerve Diseases
A family with 3460G>A and 11778G>A mutations and haplogroup analysis of Polish Leber hereditary optic neuropathy patients.
Optic Nerve Diseases
A mitochondrial DNA mutation at nucleotide pair 14459 of the NADH dehydrogenase subunit 6 gene associated with maternally inherited Leber hereditary optic neuropathy and dystonia.
Optic Nerve Diseases
A novel mitochondrial ND5 (MTND5) gene mutation giving isolated exercise intolerance.
Optic Nerve Diseases
A plasma metabolomic signature of Leber hereditary optic neuropathy showing taurine and nicotinamide deficiencies.
Optic Nerve Diseases
Allelic mutations of the fourth subunit of NADH dehydrogenase are not pathogenetically important in 11778-negative Leber hereditary optic neuropathy.
Optic Nerve Diseases
Alternative, simultaneous complex I mitochondrial DNA mutations in Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Analysis of the pathogenic human mitochondrial mutation ND1/3460, and mutations of strictly conserved residues in its vicinity, using the bacterium Paracoccus denitrificans.
Optic Nerve Diseases
Association of the mtDNA m.4171C>A/MT-ND1 mutation with both optic neuropathy and bilateral brainstem lesions.
Optic Nerve Diseases
Bioactivity and gene expression profiles of hiPSC-generated retinal ganglion cells in MT-ND4 mutated Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Biochemical features of mtDNA 14484 (ND6/M64V) point mutation associated with Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Bioenergetics of mitochondrial diseases associated with mtDNA mutations.
Optic Nerve Diseases
Bipolar cell reduction precedes retinal ganglion neuron loss in a complex 1 knockout mouse model.
Optic Nerve Diseases
Caspase-independent death of Leber's hereditary optic neuropathy cybrids is driven by energetic failure and mediated by AIF and Endonuclease G.
Optic Nerve Diseases
Catalytic activity of complex I in cell lines that possess replacement mutations in the ND genes in Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Cells bearing mutations causing Leber's hereditary optic neuropathy are sensitized to Fas-Induced apoptosis.
Optic Nerve Diseases
Changes in mitochondrial complex I activity and coenzyme Q binding site in Leber's hereditary optic neuropathy (LHON).
Optic Nerve Diseases
Cigarette toxicity triggers Leber's hereditary optic neuropathy by affecting mtDNA copy number, oxidative phosphorylation and ROS detoxification pathways.
Optic Nerve Diseases
Complex I deficiency primes Bax-dependent neuronal apoptosis through mitochondrial oxidative damage.
Optic Nerve Diseases
Complex I mutations synergize to worsen the phenotypic expression of Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Construction and detection of a novel type of recombinant human rAAV2/2-ND4.
Optic Nerve Diseases
Defective Mitochondrial Adenosine Triphosphate Production in Skeletal Muscle From Patients With Dominant Optic Atrophy Due to OPA1 Mutations.
Optic Nerve Diseases
Differentiation-specific effects of LHON mutations introduced into neuronal NT2 cells.
Optic Nerve Diseases
DNAJC30 biallelic mutations extend mitochondrial complex I-deficient phenotypes to include recessive Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Efficient mitochondrial biogenesis drives incomplete penetrance in Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Exercise Intolerance and Myoglobinuria Associated with a Novel Maternally Inherited MT-ND1 Mutation.
Optic Nerve Diseases
Fifteen novel mutations in the mitochondrial NADH dehydrogenase subunit 1, 2, 3, 4, 4L, 5 and 6 genes from Iranian patients with Leber's hereditary optic neuropathy (LHON).
Optic Nerve Diseases
Food derived respiratory complex I inhibitors modify the effect of Leber hereditary optic neuropathy mutations.
Optic Nerve Diseases
Functional analysis of lymphoblast and cybrid mitochondria containing the 3460, 11778, or 14484 Leber's hereditary optic neuropathy mitochondrial DNA mutation.
Optic Nerve Diseases
Functional consequences of the 3460-bp mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Generation of patient-specific induced pluripotent stem cells from Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Genetic and biochemical impairment of mitochondrial complex I activity in a family with Leber hereditary optic neuropathy and hereditary spastic dystonia.
Optic Nerve Diseases
Glutamate Stimulation Dysregulates AMPA Receptors-Induced Signal Transduction Pathway in Leber's Inherited Optic Neuropathy Patient-Specific hiPSC-Derived Retinal Ganglion Cells.
Optic Nerve Diseases
Glutathione depletion in antioxidant defense of differentiated NT2-LHON cybrids.
Optic Nerve Diseases
Hereditary optic neuropathies share a common mitochondrial coupling defect.
Optic Nerve Diseases
Homoplasmic and exclusive ND4 gene mutation in Japanese pedigrees with Leber's disease.
Optic Nerve Diseases
Idebenone Has Distinct Effects on Mitochondrial Respiration in Cortical Astrocytes Compared to Cortical Neurons Due to Differential NQO1 Activity.
Optic Nerve Diseases
Idebenone protects against retinal damage and loss of vision in a mouse model of Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Identification of small molecules that improve ATP synthesis defects conferred by Leber's hereditary optic neuropathy mutations.
Optic Nerve Diseases
Impaired complex I repair causes recessive Leber's hereditary optic neuropathy.
Optic Nerve Diseases
In vivo labeling of mitochondrial complex I (NADH:ubiquinone oxidoreductase) in rat brain using [(3)H]dihydrorotenone.
Optic Nerve Diseases
Induction of rapid and highly efficient expression of the human ND4 complex I subunit in the mouse visual system by self-complementary adeno-associated virus.
Optic Nerve Diseases
Inhibition of mitochondrial function induces an integrated stress response in oligodendroglia.
Optic Nerve Diseases
Intravitreal delivery of AAV-NDI1 provides functional benefit in a murine model of Leber hereditary optic neuropathy.
Optic Nerve Diseases
Leber hereditary optic neuropathy mutations in the ND6 subunit of mitochondrial complex I affect ubiquinone reduction kinetics in a bacterial model of the enzyme.
Optic Nerve Diseases
Leber's hereditary optic neuropathy (LHON) pathogenic mutations induce mitochondrial-dependent apoptotic death in transmitochondrial cells incubated with galactose medium.
Optic Nerve Diseases
Leber's hereditary optic neuropathy (LHON)-associated ND5 12338T>C mutation altered the assembly and function of complex I, apoptosis and mitophagy.
Optic Nerve Diseases
Leber's hereditary optic neuropathy and complex I deficiency in muscle.
Optic Nerve Diseases
Leber's hereditary optic neuropathy with dystonia in a Japanese family.
Optic Nerve Diseases
Leigh disease caused by the mitochondrial DNA G14459A mutation in unrelated families.
Optic Nerve Diseases
LHON/MELAS overlap mutation in ND1 subunit of mitochondrial complex I affects ubiquinone binding as revealed by modeling in Escherichia coli NDH-1.
Optic Nerve Diseases
Microphotometric analysis of NADH-tetrazolium reductase deficiency in fibroblasts of patients with Leber hereditary optic neuropathy.
Optic Nerve Diseases
Mitochondrial complex I deficiency leads to inflammation and retinal ganglion cell death in the Ndufs4 mouse.
Optic Nerve Diseases
Mitochondrial Complex I: structure, function, and implications in neurodegeneration.
Optic Nerve Diseases
Mitochondrial DNA background modulates the assembly kinetics of OXPHOS complexes in a cellular model of mitochondrial disease.
Optic Nerve Diseases
Mitochondrial DNA complex I and III mutations associated with Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Mitochondrial DNA mutations in complex I and tRNA genes in Parkinson's disease.
Optic Nerve Diseases
Mitochondrial DNA variant associated with Leber hereditary optic neuropathy and high-altitude Tibetans.
Optic Nerve Diseases
Mitochondrial Encephalopathy and Optic Neuropathy Due to m.10158 MT-ND3 Complex I Mutation Presenting in an Adult Patient: Case Report and Review of the Literature.
Optic Nerve Diseases
Mitochondrial optic neuropathy: In vivo model of neurodegeneration and neuroprotective strategies.
Optic Nerve Diseases
Mitochondrial transport mediates survival of retinal ganglion cells in affected LHON patients.
Optic Nerve Diseases
Mitophagy activation repairs Leber's hereditary optic neuropathy-associated mitochondrial dysfunction and improves cell survival.
Optic Nerve Diseases
Mouse model of optic neuropathy caused by mitochondrial complex I dysfunction.
Optic Nerve Diseases
Mutant NADH dehydrogenase subunit 4 gene delivery to mitochondria by targeting sequence-modified adeno-associated virus induces visual loss and optic atrophy in mice.
Optic Nerve Diseases
Mutation in NDUFA13/GRIM19 leads to early onset hypotonia, dyskinesia and sensorial deficiencies, and mitochondrial complex I instability.
Optic Nerve Diseases
Neuropathology of white matter disease in Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Neuroprotective effects of near-infrared light in an in vivo model of mitochondrial optic neuropathy.
Optic Nerve Diseases
Next-generation sequencing of mitochondrial targeted AAV transfer of human ND4 in mice.
Optic Nerve Diseases
Nuclear expression of mitochondrial ND4 leads to the protein assembling in complex I and prevents optic atrophy and visual loss.
Optic Nerve Diseases
Oestrogens ameliorate mitochondrial dysfunction in Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Optic neuropathy induced by reductions in mitochondrial superoxide dismutase.
Optic Nerve Diseases
Partial mitochondrial complex I inhibition induces oxidative damage and perturbs glutamate transport in primary retinal cultures. Relevance to Leber Hereditary Optic Neuropathy (LHON).
Optic Nerve Diseases
Patients with Leber hereditary optic neuropathy fail to compensate impaired oxidative phosphorylation.
Optic Nerve Diseases
Peculiar combinations of individually non-pathogenic missense mitochondrial DNA variants cause low penetrance Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Progressive optic atrophy in a retinal ganglion cell-specific mouse model of complex I deficiency.
Optic Nerve Diseases
Propagation and Selectivity of Axonal Loss in Leber Hereditary Optic Neuropathy.
Optic Nerve Diseases
Respiratory complex I dysfunction due to mitochondrial DNA mutations shifts the voltage threshold for opening of the permeability transition pore toward resting levels.
Optic Nerve Diseases
Sequence homology of NADH CoQ reductase subunit IV with nucleotide-requiring enzymes.
Optic Nerve Diseases
Sequence variations in the NDUFA1 gene encoding a subunit of complex I of the respiratory chain.
Optic Nerve Diseases
Severe impairment of complex I-driven adenosine triphosphate synthesis in leber hereditary optic neuropathy cybrids.
Optic Nerve Diseases
SOD2 gene transfer protects against optic neuropathy induced by deficiency of complex I.
Optic Nerve Diseases
The 14484 ND6 mtDNA mutation in Leber hereditary optic neuropathy does not affect fibroblast complex I activity.
Optic Nerve Diseases
The background of mitochondrial DNA haplogroup J increases the sensitivity of Leber's hereditary optic neuropathy cells to 2,5-hexanedione toxicity.
Optic Nerve Diseases
The idebenone metabolite QS10 restores electron transfer in complex I and coenzyme Q defects.
Optic Nerve Diseases
The m.3890G>A/MT-ND1 mtDNA rare pathogenic variant: Expanding clinical and MRI phenotypes.
Optic Nerve Diseases
The MELAS mutations 3946 and 3949 perturb the critical structure in a conserved loop of the ND1 subunit of mitochondrial complex I.
Optic Nerve Diseases
The metabolomic signature of Leber's hereditary optic neuropathy reveals endoplasmic reticulum stress.
Optic Nerve Diseases
The mitochondrial DNA mutation ND6*14,484C associated with leber hereditary optic neuropathy, leads to deficiency of complex I of the respiratory chain.
Optic Nerve Diseases
The mutant human ND4 subunit of complex I induces optic neuropathy in the mouse.
Optic Nerve Diseases
The ND1 gene of complex I is a mutational hot spot for Leber's hereditary optic neuropathy.
Optic Nerve Diseases
The NDUFB11 gene is not a modifier in Leber hereditary optic neuropathy.
Optic Nerve Diseases
The nuoM arg368his mutation in NADH:ubiquinone oxidoreductase from Rhodobacter capsulatus: a model for the human nd4-11778 mtDNA mutation associated with Leber's hereditary optic neuropathy.
Optic Nerve Diseases
Therapeutic applications of the TAT-mediated protein transduction system for complex I deficiency and other mitochondrial diseases.
Optic Nerve Diseases
Therapeutic strategies for Leber's hereditary optic neuropathy: A current update.
Optic Nerve Diseases
Use of transmitochondrial cybrids to assign a complex I defect to the mitochondrial DNA-encoded NADH dehydrogenase subunit 6 gene mutation at nucleotide pair 14459 that causes Leber hereditary optic neuropathy and dystonia.
Optic Nerve Diseases
XIAP Protects Retinal Ganglion Cells in the Mutant ND4 Mouse Model of Leber Hereditary Optic Neuropathy.
Optic Nerve Diseases
Yeast NDI1 improves oxidative phosphorylation capacity and increases protection against oxidative stress and cell death in cells carrying a Leber's hereditary optic neuropathy mutation.
Optic Nerve Diseases
[Molecular genetic analysis of Leber's hereditary optic neuropathy with the 3460 mutation in Japanese pedigrees]
Optic Nerve Hypoplasia
Aplasia of the retinal vessels combined with optic nerve hypoplasia, neonatal epileptic seizures, and lactic acidosis due to mitochondrial complex I deficiency.
ornithine carbamoyltransferase deficiency
Whole-exome Sequencing Helps the Diagnosis and Treatment in Children with Neurodevelopmental Delay Accompanied Unexplained Dyspnea.
Ornithine Carbamoyltransferase Deficiency Disease
Whole-exome Sequencing Helps the Diagnosis and Treatment in Children with Neurodevelopmental Delay Accompanied Unexplained Dyspnea.
Osteoarthritis
B-cell-specific mammalian target of rapamycin complex 1 activation results in severe osteoarthritis in mice.
Osteoarthritis
Chondrocyte mTORC1 activation stimulates miR-483-5p via HDAC4 in osteoarthritis progression.
Osteoarthritis
Inhibition of mTORC1 in the rat condyle subchondral bone aggravates osteoarthritis induced by the overly forward extension of the mandible.
Osteoarthritis
MTORC1 coordinates the autophagy and apoptosis signaling in articular chondrocytes in osteoarthritic temporomandibular joint.
Osteoarthritis
Synovial macrophage M1 polarisation exacerbates experimental osteoarthritis partially through R-spondin-2.
Osteochondritis Dissecans
Autophagy in farm animals: current knowledge and future challenges.
Osteoporosis
Activation of mTORC1 in B Lymphocytes Promotes Osteoclast Formation via Regulation of ?-Catenin and RANKL/OPG.
Osteoporosis
COPB2 loss of function causes a coatopathy with osteoporosis and developmental delay.
Osteosarcoma
A Humanized Bone Niche Model Reveals Bone Tissue Preservation Upon Targeting Mitochondrial Complex I in Pseudo-Orthotopic Osteosarcoma.
Osteosarcoma
Anti-cancer analogues ME-143 and ME-344 exert toxicity by directly inhibiting mitochondrial NADH: ubiquinone oxidoreductase (Complex I).
Osteosarcoma
Antibiotic anisomycin induces cell cycle arrest and apoptosis through inhibiting mitochondrial biogenesis in osteosarcoma.
Osteosarcoma
Complex I deficiency is associated with 3243G:C mitochondrial DNA in osteosarcoma cell cybrids.
Osteosarcoma
Human mitochondrial complex I assembles through the combination of evolutionary conserved modules: a framework to interpret complex I deficiencies.
Osteosarcoma
Mitochondrial outer membrane permeability change and hypersensitivity to digitonin early in staurosporine-induced apoptosis.
Osteosarcoma
Overexpression of GRIM-19 accelerates radiation-induced osteosarcoma cells apoptosis by p53 stabilization.
Osteosarcoma
Polycomb group molecule PHC3 regulates polycomb complex composition and prognosis of osteosarcoma.
Osteosarcoma
Proteome and cytoskeleton responses in osteosarcoma cells with reduced OXPHOS activity.
Osteosarcoma
The Bulk Osteosarcoma and Osteosarcoma Stem Cell Activity of a Necroptosis-Inducing Nickel(II)-Phenanthroline Complex.
Out-of-Hospital Cardiac Arrest
Mitochondrial dysfunction in adults after out-of-hospital cardiac arrest.
Ovarian Neoplasms
Bladder cancer cell-intrinsic PD-L1 signals promote mTOR and autophagy activation that can be inhibited to improve cytotoxic chemotherapy.
Ovarian Neoplasms
Co-administration of perifosine with paclitaxel synergistically induces apoptosis in ovarian cancer cells: More than just AKT inhibition.
Ovarian Neoplasms
Common alleles in candidate susceptibility genes associated with risk and development of epithelial ovarian cancer.
Ovarian Neoplasms
Development of a Long-Lived Luminescence Probe for Visualizing ?-Galactosidase in Ovarian Carcinoma Cells.
Ovarian Neoplasms
Metabolites from invasive pests inhibit mitochondrial complex II: A potential strategy for the treatment of human ovarian carcinoma?
Ovarian Neoplasms
Novel insights into the phylogenetic relationships of the endangered marsupial genus Potorous.
Ovarian Neoplasms
Phylogenetic relationships of rock-wallabies, Petrogale (Marsupialia: Macropodidae) and their biogeographic history within Australia.
Ovarian Neoplasms
Platinum-Induced Ubiquitination of Phosphorylated H2AX by RING1A Is Mediated by Replication Protein A in Ovarian Cancer.
Ovarian Neoplasms
PP242 synergizes with suberoylanilide hydroxamic acid to inhibit growth of ovarian cancer cells.
Ovarian Neoplasms
Prognostic value of GRIM-19, NF-?B and IKK2 in patients with high-grade serous ovarian cancer.
Ovarian Neoplasms
Search for cytotoxic compounds against ovarian cancer cells: Synthesis, characterization and assessment of the activity of new camphor carboxylate and camphor carboxamide silver complexes.
Ovarian Neoplasms
SIRT3 aggravates metformin-induced energy stress and apoptosis in ovarian cancer cells.
Ovarian Neoplasms
Synthesis, characterization, and reaction pathways for the formation of a GMP adduct of a cytotoxic thiocyanato ruthenium arene complex.
Ovarian Neoplasms
Zinc (II) complex with a cationic Schiff base ligand: synthesis, characterization, and biological studies.
Overnutrition
Unraveling the actions of AMP-activated protein kinase in metabolic diseases: Systemic to molecular insights.
Paget's Disease, Mammary
[Pathology of the nipple-areola complex : I. Paget's disease of the nipple, variants, and differential diagnoses].
Pancreatic Neoplasms
Modified Metformin as a More Potent Anticancer Drug: Mitochondrial Inhibition, Redox Signaling, Antiproliferative Effects and Future EPR Studies.
Pancreatic Neoplasms
Polycomb repressor complex 1 promotes gene silencing through H2AK119 mono-ubiquitination in acinar-to-ductal metaplasia and pancreatic cancer cells.
Pancreatic Neoplasms
Prostaglandin E2 activates the mTORC1 pathway through an EP4/cAMP/PKA- and EP1/Ca2+-mediated mechanism in the human pancreatic carcinoma cell line PANC-1.
Pancreatic Neoplasms
SMAD4 loss limits the vulnerability of pancreatic cancer cells to complex I inhibition via promotion of mitophagy.
Pancreatic Neoplasms
Targeting Mitochondrial Complex I Overcomes Chemoresistance in High OXPHOS Pancreatic Cancer.
Pancreatic Neoplasms
The antioxidant uncoupling protein 2 stimulates hnRNPA2/B1, GLUT1 and PKM2 expression and sensitizes pancreas cancer cells to glycolysis inhibition.
Pancreatic Neoplasms
The novel mTORC1/2 dual inhibitor INK-128 suppresses survival and proliferation of primary and transformed human pancreatic cancer cells.
Pancreatic Neoplasms
Translational reprogramming marks adaptation to asparagine restriction in cancer.
Pancreatitis
Pancreatitis associated with human immunodeficiency virus infection: a matched case-control study.
Pantothenate Kinase-Associated Neurodegeneration
Movement disorders in mitochondrial diseases.
Papillomavirus Infections
Decreased expression of GRIM-19 and its association with high-risk HPV infection in cervical squamous intraepithelial neoplasias and cancer.
Paraganglioma
Thyroid paraganglioma. Report of 3 cases and description of an immunohistochemical profile useful in the differential diagnosis with medullary thyroid carcinoma, based on complementary DNA array results.
Paralysis
Electrophysiology and pharmacology of striatal neuronal dysfunction induced by mitochondrial complex I inhibition.
Paralysis
NR2B-containing NMDA receptors promote the neurotoxic effects of 3-nitropropionic acid but not of rotenone in the striatum.
Paralysis
Short-term effects of coenzyme Q10 in progressive supranuclear palsy: a randomized, placebo-controlled trial.
Paramyxoviridae Infections
RNAi screening reveals requirement for host cell secretory pathway in infection by diverse families of negative-strand RNA viruses.
Paraparesis, Spastic
Early-onset cataracts, spastic paraparesis, and ataxia caused by a novel mitochondrial tRNAGlu (MT-TE) gene mutation causing severe complex I deficiency: a clinical, molecular, and neuropathologic study.
Paraparesis, Spastic
Recessive spastic paraparesis associated with complex I deficiency due to MTHFR mutations.
Paratuberculosis
Phagocytosis of M. paratuberculosis fails to activate expression of NADH dehydrogenase and nucleolin-related protein in bovine macrophages.
Parkinson Disease
1-Trichloromethyl-1,2,3,4-tetrahydro-beta-carboline (TaClo) Alters Cell Cycle Progression in Human Neuroblastoma Cell Lines.
Parkinson Disease
3,4-Dihydroxyphenylacetaldehyde potentiates the toxic effects of metabolic stress in PC12 cells.
Parkinson Disease
A 31P magnetic resonance spectroscopy study of mitochondrial function in skeletal muscle of patients with Parkinson's disease.
Parkinson Disease
A novel neuroprotective therapy for Parkinson's disease using a viral noncoding RNA that protects mitochondrial Complex I activity.
Parkinson Disease
A novel synthetic compound PHID (8-Phenyl-6a, 7, 8, 9, 9a, 10-hexahydro-6H-isoindolo [5, 6-g] quinoxaline-7, 9-dione) protects SH-SY5Y cells against MPP(+)-induced cytotoxicity through inhibition of reactive oxygen species generation and JNK signaling.
Parkinson Disease
A rapid method for the isolation of metabolically active mitochondria from rat neurons and astrocytes in primary culture.
Parkinson Disease
A single-subunit NADH-quinone oxidoreductase renders resistance to mammalian nerve cells against complex I inhibition.
Parkinson Disease
Abnormalities of the electron transport chain in idiopathic Parkinson's disease.
Parkinson Disease
Absence of 4,977-bp deletion of blood cell mitochondrial DNA in patients with young-onset Parkinson's disease.
Parkinson Disease
Accessory subunits are integral for assembly and function of human mitochondrial complex I.
Parkinson Disease
Activation of adenosine triphosphate-sensitive potassium channels confers protection against rotenone-induced cell death: therapeutic implications for Parkinson's disease.
Parkinson Disease
Activation of p38 and N-acetylcysteine-sensitive c-Jun NH2-terminal kinase signaling cascades is required for induction of apoptosis in Parkinson's disease cybrids.
Parkinson Disease
Age-dependent accumulation of oligomeric SNCA/?-synuclein from impaired degradation in mutant LRRK2 knockin mouse model of Parkinson disease: role for therapeutic activation of chaperone-mediated autophagy (CMA).
Parkinson Disease
AIF, reactive oxygen species, and neurodegeneration: A "complex" problem.
Parkinson Disease
Alterations in the Mitochondrial Proteome of Neuroblastoma Cells in Response to Complex 1 Inhibition.
Parkinson Disease
Altered calcium homeostasis in cells transformed by mitochondria from individuals with Parkinson's disease.
Parkinson Disease
Altered dopamine metabolism and increased vulnerability to MPTP in mice with partial deficiency of mitochondrial complex I in dopamine neurons.
Parkinson Disease
Altered mitochondrial function, iron metabolism and glutathione levels in Parkinson's disease.
Parkinson Disease
Alternative mitochondrial quality control mediated by extracellular release.
Parkinson Disease
An in vitro model of Parkinson's disease: linking mitochondrial impairment to altered alpha-synuclein metabolism and oxidative damage.
Parkinson Disease
Analysis of the assembly profiles for mitochondrial- and nuclear-DNA-encoded subunits into complex I.
Parkinson Disease
Anatomic and disease specificity of NADH CoQ1 reductase (complex I) deficiency in Parkinson's disease.
Parkinson Disease
Anti-oxidant polydatin (piceid) protects against substantia nigral motor degeneration in multiple rodent models of Parkinson's disease.
Parkinson Disease
Apoptosis-inducing factor deficiency sensitizes dopaminergic neurons to parkinsonian neurotoxins.
Parkinson Disease
Assay of [3H]dihydrorotenone binding to complex I in intact human platelets.
Parkinson Disease
Astrocytes protect MN9D neuronal cells against rotenone-induced oxidative stress by a glutathione-dependent mechanism.
Parkinson Disease
ATP-sensitive potassium channels in dopaminergic neurons: transducers of mitochondrial dysfunction.
Parkinson Disease
Bacopa monnieri Phytochemicals Mediated Synthesis of Platinum Nanoparticles and Its Neurorescue Effect on 1-Methyl 4-Phenyl 1,2,3,6 Tetrahydropyridine-Induced Experimental Parkinsonism in Zebrafish.
Parkinson Disease
Bilateral upregulation of ?-synuclein expression in the mouse substantia nigra by intracranial rotenone treatment.
Parkinson Disease
Biochemical analysis of cybrids expressing mitochondrial DNA from Contursi kindred Parkinson's subjects.
Parkinson Disease
Bioenergetics and the formation of mitochondrial reactive oxygen species.
Parkinson Disease
Brain mitochondria catalyze the oxidation of 7-(2-aminoethyl)-3,4-dihydro-5-hydroxy-2H-1,4-benzothiazine-3-carboxyli c acid (DHBT-1) to intermediates that irreversibly inhibit complex I and scavenge glutathione: potential relevance to the pathogenesis of Parkinson's disease.
Parkinson Disease
Brain, skeletal muscle and platelet homogenate mitochondrial function in Parkinson's disease.
Parkinson Disease
Brain-derived neurotrophic factor-mediated effects on mitochondrial respiratory coupling and neuroprotection share the same molecular signalling pathways.
Parkinson Disease
Ca2+-induced oxidative stress in brain mitochondria treated with the respiratory chain inhibitor rotenone.
Parkinson Disease
Caffeic acid improves locomotor activity and lessens inflammatory burden in a mouse model of rotenone-induced nigral neurodegeneration: Relevance to Parkinson's disease therapy.
Parkinson Disease
Carbidopa/levodopa and selegiline do not affect platelet mitochondrial function in early parkinsonism.
Parkinson Disease
Cardiolipin promotes electron transport between ubiquinone and complex I to rescue PINK1 deficiency.
Parkinson Disease
Caspase-3 activation induced by inhibition of mitochondrial complex I is facilitated by glycogen synthase kinase-3beta and attenuated by lithium.
Parkinson Disease
Celastrol from 'Thunder God Vine' protects SH-SY5Y cells through the preservation of mitochondrial function and inhibition of p38 MAPK in a rotenone model of Parkinson's disease.
Parkinson Disease
Characterization and time course of MPP+ -induced apoptosis in human SH-SY5Y neuroblastoma cells.
Parkinson Disease
Chloramphenicol Mitigates Oxidative Stress by Inhibiting Translation of Mitochondrial Complex I in Dopaminergic Neurons of Toxin-Induced Parkinson's Disease Model.
Parkinson Disease
Chronic MPTP treatment reproduces in baboons the differential vulnerability of mesencephalic dopaminergic neurons observed in Parkinson's disease.
Parkinson Disease
Chronic reduction in complex I function alters calcium signaling in SH-SY5Y neuroblastoma cells.
Parkinson Disease
Chronic systemic complex I inhibition induces a hypokinetic multisystem degeneration in rats.
Parkinson Disease
Chronic systemic pesticide exposure reproduces features of Parkinson's disease.
Parkinson Disease
Chronic, low-dose rotenone reproduces Lewy neurites found in early stages of Parkinson's disease, reduces mitochondrial movement and slowly kills differentiated SH-SY5Y neural cells.
Parkinson Disease
Coenzyme Q10 levels correlate with the activities of complexes I and II/III in mitochondria from parkinsonian and nonparkinsonian subjects.
Parkinson Disease
Coenzyme Q10 supplementation provides mild symptomatic benefit in patients with Parkinson's disease.
Parkinson Disease
Combined PET/MRS brain studies show dynamic and long-term physiological changes in a primate model of Parkinson disease.
Parkinson Disease
Complex I Controls Mitochondrial and Plasma Membrane Potentials in Nerve Terminals.
Parkinson Disease
Complex I deficiency primes Bax-dependent neuronal apoptosis through mitochondrial oxidative damage.
Parkinson Disease
Complex I inhibitors induce dose-dependent apoptosis in PC12 cells: relevance to Parkinson's disease.
Parkinson Disease
Complex I polymorphisms, bigenomic heterogeneity, and family history in Virginians with Parkinson's disease.
Parkinson Disease
Complex I syndrome in striatum and frontal cortex in a rat model of Parkinson disease.
Parkinson Disease
Conditional deletion of Ndufs4 in dopaminergic neurons promotes Parkinson's disease-like non-motor symptoms without loss of dopamine neurons.
Parkinson Disease
Constitutive expression and functional characterization of mitochondrial glutaredoxin (Grx2) in mouse and human brain.
Parkinson Disease
Controversies on new animal models of Parkinson's disease pro and con: the rotenone model of Parkinson's disease (PD).
Parkinson Disease
Cyclosporin inhibition of apoptosis induced by mitochondrial complex I toxins.
Parkinson Disease
Cytochrome c release from rat brain mitochondria is proportional to the mitochondrial functional deficit: implications for apoptosis and neurodegenerative disease.
Parkinson Disease
D-beta-hydroxybutyrate protects neurons in models of Alzheimer's and Parkinson's disease.
Parkinson Disease
Da-Bu-Yin-Wan and Qian-Zheng-San, two traditional Chinese herbal formulas, up-regulate the expression of mitochondrial subunit NADH dehydrogenase 1 synergistically in the mice model of Parkinson's disease.
Parkinson Disease
Deficiencies in complex I subunits of the respiratory chain in Parkinson's disease.
Parkinson Disease
Deficits in a tricarboxylic acid cycle enzyme in brains from patients with Parkinson's disease.
Parkinson Disease
Dementia in Parkinson's disease is associated with enhanced mitochondrial complex I deficiency.
Parkinson Disease
Depletion of glutathione does not affect electron transport chain complex activity in brain mitochondria: Implications for Parkinson disease and postmortem studies.
Parkinson Disease
Depolarization of in situ mitochondria due to hydrogen peroxide-induced oxidative stress in nerve terminals: inhibition of alpha-ketoglutarate dehydrogenase.
Parkinson Disease
Detection of nitrosyl complexes in human substantia nigra, in relation to Parkinson's disease.
Parkinson Disease
Detoxified Extract of Rhus verniciflua Stokes Inhibits Rotenone-Induced Apoptosis in Human Dopaminergic Cells, SH-SY5Y.
Parkinson Disease
Differential Effects of Yeast NADH Dehydrogenase (Ndi1) Expression on Mitochondrial Function and Inclusion Formation in a Cell Culture Model of Sporadic Parkinson's Disease.
Parkinson Disease
Direct regulation of complex I by mitochondrial MEF2D is disrupted in a mouse model of Parkinson disease and in human patients.
Parkinson Disease
Disrupted mitochondrial electron transport function increases expression of anti-apoptotic bcl-2 and bcl-X(L) proteins in SH-SY5Y neuroblastoma and in Parkinson disease cybrid cells through oxidative stress.
Parkinson Disease
Distinct mechanisms of neurodegeneration induced by chronic complex I inhibition in dopaminergic and non-dopaminergic cells.
Parkinson Disease
DJ-1 deficiency in astrocytes selectively enhances mitochondrial Complex I inhibitor-induced neurotoxicity.
Parkinson Disease
Dopamine gene therapy for Parkinson's disease in a nonhuman primate without associated dyskinesia.
Parkinson Disease
Dopamine is involved in selectivity of dopaminergic neuronal death by rotenone.
Parkinson Disease
Dopamine Release Suppression Dependent on an Increase of Intracellular Ca(2+) Contributed to Rotenone-induced Neurotoxicity in PC12 Cells.
Parkinson Disease
Dopamine-derived dopaminochrome promotes H(2)O(2) release at mitochondrial complex I: stimulation by rotenone, control by Ca(2+), and relevance to Parkinson disease.
Parkinson Disease
Downregulation of PMCA2 increases the vulnerability of midbrain neurons to mitochondrial complex I inhibition.
Parkinson Disease
Dysfunction of mitochondrial complex I and the proteasome: interactions between two biochemical deficits in a cellular model of Parkinson's disease.
Parkinson Disease
Early life exposure to permethrin: a progressive animal model of Parkinson's disease.
Parkinson Disease
Echinacoside selectively rescues complex I inhibition-induced mitochondrial respiratory impairment via enhancing complex II activity.
Parkinson Disease
Effect of antioxidants on L-glutamate and N-methyl-4-phenylpyridinium ion induced-neurotoxicity in PC12 cells.
Parkinson Disease
Effect of mitochondrial complex I inhibition on Fe-S cluster protein activity.
Parkinson Disease
Effects of epigallocatechin gallate on rotenone-injured murine brain cultures.
Parkinson Disease
Effects of glutamate and alpha2-noradrenergic receptor antagonists on the development of neurotoxicity produced by chronic rotenone in rats.
Parkinson Disease
Effects of Treadmill Exercise on Neural Mitochondrial Functions in Parkinson's Disease: A Systematic Review of Animal Studies.
Parkinson Disease
Efficacy of catalpol as protectant against oxidative stress and mitochondrial dysfunction on rotenone-induced toxicity in mice brain.
Parkinson Disease
Electron transfer complexes I and IV of platelets are abnormal in Parkinson's disease but normal in Parkinson-plus syndromes.
Parkinson Disease
Electrophysiology and pharmacology of striatal neuronal dysfunction induced by mitochondrial complex I inhibition.
Parkinson Disease
Elevated reactive oxygen species and antioxidant enzyme activities in animal and cellular models of Parkinson's disease.
Parkinson Disease
Energy thresholds in brain mitochondria. Potential involvement in neurodegeneration.
Parkinson Disease
Engrailed protects mouse midbrain dopaminergic neurons against mitochondrial complex I insults.
Parkinson Disease
Evidence for mitochondrial dysfunction in Parkinson's disease--a critical appraisal.
Parkinson Disease
Extracellular accumulation of nitric oxide, hydrogen peroxide, and glutamate in astrocytic cultures following glutathione depletion, complex I inhibition, and/or lipopolysaccharide-induced activation.
Parkinson Disease
Flotillin-1 in the substantia nigra of the Parkinson brain and a predominant localization in catecholaminergic nerves in the rat brain.
Parkinson Disease
Free radical scavengers protect dopaminergic cell lines from apoptosis induced by complex I inhibitors.
Parkinson Disease
Gene expression profiling of rotenone-mediated cortical neuronal death: Evidence for inhibition of ubiquitin-proteasome system and autophagy-lysosomal pathway, and dysfunction of mitochondrial and calcium signaling.
Parkinson Disease
Generation of reactive oxygen species by mitochondrial complex I: implications in neurodegeneration.
Parkinson Disease
Genetic reduction of mitochondrial complex I function does not lead to loss of dopamine neurons in vivo.
Parkinson Disease
Genetic variation of the mitochondrial complex I subunit NDUFV2 and Parkinson disease.
Parkinson Disease
Genotype in the 24-kDa subunit gene (NDUFV2) of mitochondrial complex I and susceptibility to Parkinson disease.
Parkinson Disease
Ghrelin protects MES23.5 cells against rotenone via inhibiting mitochondrial dysfunction and apoptosis.
Parkinson Disease
Glutaredoxin 2 catalyzes the reversible oxidation and glutathionylation of mitochondrial membrane thiol proteins: implications for mitochondrial redox regulation and antioxidant DEFENSE.
Parkinson Disease
Glutaredoxin is essential for maintenance of brain mitochondrial complex I: studies with MPTP.
Parkinson Disease
Glutathione depletion in a midbrain-derived immortalized dopaminergic cell line results in limited tyrosine nitration of mitochondrial complex I subunits: implications for Parkinson's disease.
Parkinson Disease
Glutathione depletion in PC12 results in selective inhibition of mitochondrial complex I activity. Implications for Parkinson's disease.
Parkinson Disease
Glutathione depletion resulting in selective mitochondrial complex I inhibition in dopaminergic cells is via an NO-mediated pathway not involving peroxynitrite: implications for Parkinson's disease.
Parkinson Disease
Glycogen synthase kinase-3? activation mediates rotenone-induced cytotoxicity with the involvement of microtubule destabilization.
Parkinson Disease
Hexokinase II gene transfer protects against neurodegeneration in the rotenone and MPTP mouse models of Parkinson's disease.
Parkinson Disease
High expression of nicotinamide N-methyltransferase in patients with idiopathic Parkinson's disease.
Parkinson Disease
High frequency of mitochondrial complex I mutations in Parkinson's disease and aging.
Parkinson Disease
Identification of genes associated with the effect of inflammation on the neurotransmission of vascular smooth muscle cell.
Parkinson Disease
Identification of novel proteins affected by rotenone in mitochondria of dopaminergic cells.
Parkinson Disease
Imaging mass cytometry reveals generalised deficiency in OXPHOS complexes in Parkinson's disease.
Parkinson Disease
Immunocytochemical characterization of the mitochondrially encoded ND1 subunit of complex I (NADH : ubiquinone oxidoreductase) in rat brain.
Parkinson Disease
Immunohistochemical studies on complexes I, II, III, and IV of mitochondria in Parkinson's disease.
Parkinson Disease
Impaired oxidative decarboxylation of pyruvate in fibroblasts from patients with Parkinson's disease.
Parkinson Disease
Implications of enzyme deficiencies on mitochondrial energy metabolism and reactive oxygen species formation of neurons involved in rotenone-induced Parkinson's disease: a model-based analysis.
Parkinson Disease
In vivo complementation of complex I by the yeast Ndi1 enzyme. Possible application for treatment of Parkinson disease.
Parkinson Disease
In vivo labeling of mitochondrial complex I (NADH:ubiquinone oxidoreductase) in rat brain using [(3)H]dihydrorotenone.
Parkinson Disease
Increased striatal dopamine turnover following acute administration of rotenone to mice.
Parkinson Disease
Increased vulnerability to rotenone-induced neurotoxicity in ceruloplasmin-deficient mice.
Parkinson Disease
Indices of oxidative stress and mitochondrial function in individuals with incidental Lewy body disease.
Parkinson Disease
Inhibition of alpha-ketoglutarate dehydrogenase by isoquinoline derivatives structurally related to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP).
Parkinson Disease
Inhibition of complex I by isoquinoline derivatives structurally related to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP).
Parkinson Disease
Inhibition of complex I by neuroleptics in normal human brain cortex parallels the extrapyramidal toxicity of neuroleptics.
Parkinson Disease
Inhibition of mitochondrial respiratory complex I by nitric oxide, peroxynitrite and S-nitrosothiols.
Parkinson Disease
Initiation of neuronal damage by complex I deficiency and oxidative stress in Parkinson's disease.
Parkinson Disease
Intermittent Fasting Applied in Combination with Rotenone Treatment Exacerbates Dopamine Neurons Degeneration in Mice.
Parkinson Disease
Intranasal Administration of Rotenone Reduces GABAergic Inhibition in the Mouse Insular Cortex Leading to Impairment of LTD and Conditioned Taste Aversion Memory.
Parkinson Disease
Involvement of ERK1/2 pathway in neuroprotective effects of pyrroloquinoline quinine against rotenone-induced SH-SY5Y cell injury.
Parkinson Disease
Iron induced oxidative stress and mitochondrial dysfunction: relevance to Parkinson's disease.
Parkinson Disease
Irreversible inhibition of mitochondrial complex I by 1-methyl-4-phenylpyridinium: evidence for free radical involvement.
Parkinson Disease
Irreversible inhibition of mitochondrial complex I by 7-(2-aminoethyl)-3,4-dihydro-5-hydroxy-2H-1,4-benzothiazine-3-carboxyli c acid (DHBT-1): a putative nigral endotoxin of relevance to Parkinson's disease.
Parkinson Disease
Isolated cerebral and cerebellar mitochondria produce free radicals when exposed to elevated CA2+ and Na+: implications for neurodegeneration.
Parkinson Disease
L-dihydroxyphenylalanine and complex I deficiency in Parkinson's disease brain.
Parkinson Disease
Loss of mitochondrial complex I activity potentiates dopamine neuron death induced by microtubule dysfunction in a Parkinson's disease model.
Parkinson Disease
Loss-of-Function NUBPL Mutation May Link Parkinson's Disease to Recessive Complex I Deficiency.
Parkinson Disease
Low dose rotenone treatment causes selective transcriptional activation of cell death related pathways in dopaminergic neurons in vivo.
Parkinson Disease
Low platelet mitochondrial complex I and complex II/III activity in early untreated Parkinson's disease.
Parkinson Disease
Matrilineal inheritance of complex I dysfunction in a multigenerational Parkinson's disease family.
Parkinson Disease
Mechanism of toxicity of pesticides acting at complex I: relevance to environmental etiologies of Parkinson's disease.
Parkinson Disease
Melatonin protects against 6-OHDA-induced neurotoxicity in rats: a role for mitochondrial complex I activity.
Parkinson Disease
Mesencephalic complex I deficiency does not correlate with parkinsonism in mitochondrial DNA maintenance disorders.
Parkinson Disease
Metabolic enzyme expression in dopaminergic neurons in Parkinson's disease: an in situ hybridization study.
Parkinson Disease
Metabolic stress in PC12 cells induces the formation of the endogenous dopaminergic neurotoxin, 3,4-dihydroxyphenylacetaldehyde.
Parkinson Disease
Metformin, besides exhibiting strong in vivo anti-inflammatory properties, increases mptp-induced damage to the nigrostriatal dopaminergic system.
Parkinson Disease
Methylphenylpyridium ion (MPP+) enhances glutamate-induced cytotoxicity against dopaminergic neurons in cultured rat mesencephalon.
Parkinson Disease
Methylpyridinium (MPP(+))- and nerve growth factor-induced changes in pro- and anti-apoptotic signaling pathways in SH-SY5Y neuroblastoma cells.
Parkinson Disease
Mitochondria deficient in complex I activity are depolarized by hydrogen peroxide in nerve terminals: relevance to Parkinson's disease.
Parkinson Disease
Mitochondria in neurodegenerative disorders: regulation of the redox state and death signaling leading to neuronal death and survival.
Parkinson Disease
Mitochondrial Complex 1, Sigma 1, and Synaptic Vesicle 2A in Early Drug-Naive Parkinson's Disease.
Parkinson Disease
Mitochondrial Complex I Activity is Conditioned by Supercomplex I-III2-IV Assembly in Brain Cells: Relevance for Parkinson's Disease.
Parkinson Disease
Mitochondrial complex I and II activities of lymphocytes and platelets in Parkinson's disease.
Parkinson Disease
Mitochondrial complex I deficiency in Parkinson's disease.
Parkinson Disease
Mitochondrial complex I gene variant associated with early age at onset in spinocerebellar ataxia type 2.
Parkinson Disease
Mitochondrial complex I inhibition depletes plasma testosterone in the rotenone model of Parkinson's disease.
Parkinson Disease
Mitochondrial complex I inhibition in Parkinson's disease: how can curcumin protect mitochondria?
Parkinson Disease
Mitochondrial complex I inhibition is not required for dopaminergic neuron death induced by rotenone, MPP+, or paraquat.
Parkinson Disease
Mitochondrial complex I inhibitor rotenone-induced toxicity and its potential mechanisms in Parkinson's disease models.
Parkinson Disease
Mitochondrial complex I polymorphism and cigarette smoking in Parkinson's disease.
Parkinson Disease
Mitochondrial Complex I Reversible S-Nitrosation Improves Bioenergetics and Is Protective in Parkinson's Disease.
Parkinson Disease
Mitochondrial complex I, II/III, and IV activities in familial and sporadic Parkinson's disease.
Parkinson Disease
Mitochondrial Complex I: structure, function, and implications in neurodegeneration.
Parkinson Disease
Mitochondrial DNA mutations in complex I and tRNA genes in Parkinson's disease.
Parkinson Disease
Mitochondrial DNA polymorphisms and haplogroups in Parkinson's disease and control individuals with a similar genetic background.
Parkinson Disease
Mitochondrial DNA polymorphisms as risk factors for Parkinson's disease and Parkinson's disease dementia.
Parkinson Disease
Mitochondrial DNA transmission of the mitochondrial defect in Parkinson's disease.
Parkinson Disease
Mitochondrial dysfunction and possible treatments in Parkinson's disease--a review.
Parkinson Disease
Mitochondrial dysfunction in Parkinson's disease: pathogenesis and neuroprotection.
Parkinson Disease
Mitochondrial dysfunction triggers the pathogenesis of Parkinson's disease in neuronal C/EBP? transgenic mice.
Parkinson Disease
Mitochondrial function in Parkinson's disease. The Royal Kings and Queens Parkinson's Disease Research Group.
Parkinson Disease
Mitochondrial function, GSH and iron in neurodegeneration and Lewy body diseases.
Parkinson Disease
Mitochondrial Import and Accumulation of {alpha}-Synuclein Impair Complex I in Human Dopaminergic Neuronal Cultures and Parkinson Disease Brain.
Parkinson Disease
Mitochondrial Inhibitor Models of Huntington's Disease and Parkinson's Disease Induce Zinc Accumulation and Are Attenuated by Inhibition of Zinc Neurotoxicity in vitro or in vivo.
Parkinson Disease
Mitochondrial ND1 sequence analysis and association of the T4216C mutation with Parkinson's disease.
Parkinson Disease
Mitochondrial polymorphisms significantly reduce the risk of Parkinson disease.
Parkinson Disease
Mitochondrial respiratory chain disorganization in Parkinson's disease-relevant PINK1 and DJ1 mutants.
Parkinson Disease
Mitochondrial respiratory enzyme function and superoxide dismutase activity following brain glutathione depletion in the rat.
Parkinson Disease
Mitochondrial toxins in models of neurodegenerative diseases. II: Elevated zif268 transcription and independent temporal regulation of striatal D1 and D2 receptor mRNAs and D1 and D2 receptor-binding sites in C57BL/6 mice during MPTP treatment.
Parkinson Disease
Mitochondrial variations in the MT-ND4 and MT-TL1 genes are associated with male infertility.
Parkinson Disease
Modulation of connexin 43 in rotenone-induced model of Parkinson's disease.
Parkinson Disease
Molecular characterization of dopamine-derived quinones reactivity toward NADH and glutathione: implications for mitochondrial dysfunction in Parkinson disease.
Parkinson Disease
Molecular pathways of programmed cell death in experimental Parkinson's disease.
Parkinson Disease
Monitoring mitochondrial dynamics and complex I dysfunction in neurons: implications for Parkinson's disease.
Parkinson Disease
Multiple metabolic changes mediate the response of Caenorhabditis elegans to the complex I inhibitor rotenone.
Parkinson Disease
Multivariate meta-analyses of mitochondrial complex I and IV in major depressive disorder, bipolar disorder, schizophrenia, Alzheimer disease, and Parkinson disease.
Parkinson Disease
Muscle respiratory chain enzyme activities in parkinson's disease and in multisystem extrapyramidal disorders with parkinsonism as the main clinical feature.
Parkinson Disease
N-acetylcysteine elicited increase in complex I activity in synaptic mitochondria from aged mice: implications for treatment of Parkinson's disease.
Parkinson Disease
NAD+ Regeneration Rescues Lifespan, but Not Ataxia, in a Mouse Model of Brain Mitochondrial Complex I Dysfunction.
Parkinson Disease
Naringin Protects against Rotenone-induced Apoptosis in Human Neuroblastoma SH-SY5Y Cells.
Parkinson Disease
Neurobehavioural Changes in a Hemiparkinsonian Rat Model Induced by Rotenone.
Parkinson Disease
Neuroleptic medications inhibit complex I of the electron transport chain.
Parkinson Disease
Neuronal complex I deficiency occurs throughout the Parkinson's disease brain, but is not associated with neurodegeneration or mitochondrial DNA damage.
Parkinson Disease
Neuroprotection by valproic acid in an intrastriatal rotenone model of Parkinson's disease.
Parkinson Disease
Neuroprotective effects of ginsenoside-Rg1 in primary nigral neurons against rotenone toxicity.
Parkinson Disease
Neuroprotective efficacy of Bacopa monnieri against rotenone induced oxidative stress and neurotoxicity in Drosophila melanogaster.
Parkinson Disease
Nicotinamide N-methyltransferase catalyses the N-methylation of the endogenous ?-carboline norharman: evidence for a novel detoxification pathway.
Parkinson Disease
Nitrosylation and nitration of mitochondrial complex I in Parkinson's disease.
Parkinson Disease
Normal mitochondrial genome in brain from patients with Parkinson's disease and complex I defect.
Parkinson Disease
Novel (Hetero)arylalkenyl propargylamine compounds are protective in toxin-induced models of Parkinson's disease.
Parkinson Disease
Novel mutations of mitochondrial complex I in pathologically proven Parkinson disease.
Parkinson Disease
Obligatory role for complex I inhibition in the dopaminergic neurotoxicity of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP).
Parkinson Disease
Optic atrophy 1 mediates mitochondria remodeling and dopaminergic neurodegeneration linked to complex I deficiency.
Parkinson Disease
Origin and functional consequences of the complex I defect in Parkinson's disease.
Parkinson Disease
Oxidative damage to mitochondrial complex I due to peroxynitrite: identification of reactive tyrosines by mass spectrometry.
Parkinson Disease
Oxidative metabolites of 5-S-cysteinyldopamine inhibit the alpha-ketoglutarate dehydrogenase complex: possible relevance to the pathogenesis of Parkinson's disease.
Parkinson Disease
Oxidative stress in skin fibroblasts cultures from patients with Parkinson's disease.
Parkinson Disease
Oxidative Stress Indices in Parkinson's Disease : Biochemical Determination.
Parkinson Disease
PACAP protects neuronal differentiated PC12 cells against the neurotoxicity induced by a mitochondrial complex I inhibitor, rotenone.
Parkinson Disease
Parkinson's disease and mitochondrial complex I: a perspective on the Ndi1 therapy.
Parkinson Disease
Parkinson's disease and mitochondrial DNA NADH dehydrogenase subunit 1 nucleotides 3337-3340: study in a population from the central region of Portugal (Coimbra).
Parkinson Disease
Parkinson's disease brain mitochondrial complex I has oxidatively damaged subunits and is functionally impaired and misassembled.
Parkinson Disease
Parkinson's disease in relation to pesticide exposure and nuclear encoded mitochondrial complex I gene variants.
Parkinson Disease
Parkinson's disease mutations in PINK1 result in decreased Complex I activity and deficient synaptic function.
Parkinson Disease
Parkinson's Disease-Associated Pathogenic VPS35 Mutation Causes Complex I Deficits.
Parkinson Disease
Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage.
Parkinson Disease
Partial inhibition of complex I activity increases Ca-independent glutamate release rates from depolarized synaptosomes.
Parkinson Disease
PCR analysis of platelet mtDNA: lack of specific changes in Parkinson's disease.
Parkinson Disease
Persistent S-nitrosation of complex I and other mitochondrial membrane proteins by S-nitrosothiols but not nitric oxide or peroxynitrite: implications for the interaction of nitric oxide with mitochondria.
Parkinson Disease
PET Imaging of Mitochondrial Complex I with 18F-BCPP-EF in the Brains of MPTP-Treated Monkeys.
Parkinson Disease
Platelet mitochondrial function in Parkinson's disease. The Royal Kings and Queens Parkinson Disease Research Group.
Parkinson Disease
Possible involvement of both mitochondria- and endoplasmic reticulum-dependent caspase pathways in rotenone-induced apoptosis in human neuroblastoma SH-SY5Y cells.
Parkinson Disease
Possible involvement of Ca2+ signaling in rotenone-induced apoptosis in human neuroblastoma SH-SY5Y cells.
Parkinson Disease
PPAR? activation rescues mitochondrial function from inhibition of complex I and loss of PINK1.
Parkinson Disease
Pramipexole reduces reactive oxygen species production in vivo and in vitro and inhibits the mitochondrial permeability transition produced by the parkinsonian neurotoxin methylpyridinium ion.
Parkinson Disease
Production of reactive oxygen species in brain mitochondria: contribution by electron transport chain and non-electron transport chain sources.
Parkinson Disease
Protection against MPP+ neurotoxicity in cerebellar granule cells by antioxidants.
Parkinson Disease
Protection by the NDI1 Gene against Neurodegeneration in a Rotenone Rat Model of Parkinson's Disease.
Parkinson Disease
Protective effect of melatonin on rotenone plus Ca2+-induced mitochondrial oxidative stress and PC12 cell death.
Parkinson Disease
Protective effect of metformin against rotenone-induced parkinsonism in mice.
Parkinson Disease
Protective effects of synthetic kynurenines on 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-induced parkinsonism in mice.
Parkinson Disease
Protective Role of rAAV-NDI1, Serotype 5, in an Acute MPTP Mouse Parkinson's Model.
Parkinson Disease
Quantification of NADH:ubiquinone oxidoreductase (complex I) content in biological samples.
Parkinson Disease
Quantification of oxidative phosphorylation enzymes after blue native electrophoresis and two-dimensional resolution: normal complex I protein amounts in Parkinson's disease conflict with reduced catalytic activities.
Parkinson Disease
Quantitative autoradiography of dihydrorotenone binding to complex I of the electron transport chain.
Parkinson Disease
Quantitative mapping of reversible mitochondrial Complex I cysteine oxidation in a Parkinson disease mouse model.
Parkinson Disease
Quantitative relationship between inhibition of respiratory complexes and formation of reactive oxygen species in isolated nerve terminals.
Parkinson Disease
Quantitative study of mitochondrial complex I in platelets of parkinsonian patients.
Parkinson Disease
Randomized, double-blind, placebo-controlled pilot trial of reduced coenzyme Q10 for Parkinson's disease.
Parkinson Disease
Reduced NADH coenzyme Q dehydrogenase activity in platelets of Parkinson's disease, but not Parkinson plus patients, from an Indian population.
Parkinson Disease
Regulation of a distinct activated RIPK1 intermediate bridging complex I and complex II in TNF?-mediated apoptosis.
Parkinson Disease
Respiratory chain and mitochondrial deoxyribonucleic acid in blood cells from patients with focal and generalized dystonia.
Parkinson Disease
Respiratory chain and mitochondrial DNA in muscle and brain in Parkinson's disease patients.
Parkinson Disease
Reversible inhibition of mitochondrial complex I activity following chronic dopaminergic glutathione depletion in vitro: implications for Parkinson's disease.
Parkinson Disease
Role of mitochondrial dysfunction in Parkinson's disease: Implications for treatment.
Parkinson Disease
Rotenone and CCCP inhibit tyrosine hydroxylation in rat striatal tissue slices.
Parkinson Disease
Rotenone Decreases Intracellular Aldehyde Dehydrogenase Activity: Implications for the Pathogenesis of Parkinson Disease.
Parkinson Disease
Rotenone destroys dopaminergic neurons and induces parkinsonian symptoms in rats.
Parkinson Disease
Rotenone exerts similar stimulatory effects on H2O2 production by isolated brain mitochondria from young-adult and old rats.
Parkinson Disease
Rotenone increases glutamate-induced dopamine release but does not affect hydroxyl-free radical formation in rat striatum.
Parkinson Disease
Rotenone induces apoptosis via activation of bad in human dopaminergic SH-SY5Y cells.
Parkinson Disease
Rotenone induces oxidative stress and dopaminergic neuron damage in organotypic substantia nigra cultures.
Parkinson Disease
Rotenone induces reductive stress and triacylglycerol deposition in C2C12 cells.
Parkinson Disease
Rotenone induction of hydrogen peroxide inhibits mTOR-mediated S6K1 and 4E-BP1/eIF4E pathways, leading to neuronal apoptosis.
Parkinson Disease
Rotenone-induced necrosis in insect cells via the cytoplasmic membrane damage and mitochondrial dysfunction.
Parkinson Disease
Rotenone-mediated changes in intracellular coenzyme A thioester levels: implications for mitochondrial dysfunction.
Parkinson Disease
Salidroside Protects Dopaminergic Neurons by Preserving Complex I Activity via DJ-1/Nrf2-Mediated Antioxidant Pathway.
Parkinson Disease
Salidroside Protects Dopaminergic Neurons by Regulating the Mitochondrial MEF2D-ND6 Pathway in the MPTP/MPP+ -Induced Model of Parkinson's Disease.
Parkinson Disease
Sequence analysis of two nuclear encoded subunits (10 and 51 kDa) of mitochondrial complex I in Parkinson disease.
Parkinson Disease
Sex Differences in Rotenone Sensitivity Reflect the Male-to-Female Ratio in Human Parkinson's Disease Incidence.
Parkinson Disease
Silencing of peroxiredoxin 3 and peroxiredoxin 5 reveals the role of mitochondrial peroxiredoxins in the protection of human neuroblastoma SH-SY5Y cells toward MPP(+).
Parkinson Disease
Sodium salicylate protects against rotenone-induced Parkinsonism in rats.
Parkinson Disease
Spare respiratory capacity rather than oxidative stress regulates glutamate excitotoxicity after partial respiratory inhibition of mitochondrial complex I with rotenone.
Parkinson Disease
Subthalamic nucleus-mediated excitotoxicity in Parkinson's disease: a target for neuroprotection.
Parkinson Disease
Synergistic effects of melatonin and deprenyl against MPTP-induced mitochondrial damage and DA depletion.
Parkinson Disease
Systemic administration of rotenone produces selective damage in the striatum and globus pallidus, but not in the substantia nigra.
Parkinson Disease
Targeting the Mitochondrial Permeability Transition Pore to Prevent Age-Associated Cell Damage and Neurodegeneration.
Parkinson Disease
Tetrahydroxystilbene glucoside protects human neuroblastoma SH-SY5Y cells against MPP+-induced cytotoxicity.
Parkinson Disease
The effect of endogenous dopamine in rotenone-induced toxicity in PC12 cells.
Parkinson Disease
The isomers of thioctic acid alter C-deoxyglucose incorporation in rat basal ganglia.
Parkinson Disease
The mitochondrial complex I inhibitor annonacin is toxic to mesencephalic dopaminergic neurons by impairment of energy metabolism.
Parkinson Disease
The PARK10 gene USP24 is a negative regulator of autophagy and ULK1 protein stability.
Parkinson Disease
The parkinsonian neurotoxin MPP+ opens the mitochondrial permeability transition pore and releases cytochrome c in isolated mitochondria via an oxidative mechanism.
Parkinson Disease
The parkinsonism-inducing drug 1-methyl-4-phenylpyridinium triggers intracellular dopamine oxidation. A novel mechanism of toxicity.
Parkinson Disease
The role of glycolysis and gluconeogenesis in the cytoprotection of neuroblastoma cells against 1-methyl 4-phenylpyridinium ion toxicity.
Parkinson Disease
The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification.
Parkinson Disease
The transgenic overexpression of alpha-synuclein and not its related pathology associates with complex I inhibition.
Parkinson Disease
Transcriptional analysis of multiple brain regions in Parkinson's disease supports the involvement of specific protein processing, energy metabolism, and signaling pathways, and suggests novel disease mechanisms.
Parkinson Disease
Transcriptome analysis of a rotenone model of parkinsonism reveals complex I-tied and -untied toxicity mechanisms common to neurodegenerative diseases.
Parkinson Disease
Treatment of Parkinson disease with diet-induced hyperketonemia: a feasibility study.
Parkinson Disease
Two molecular pathways initiate mitochondria-dependent dopaminergic neurodegeneration in experimental Parkinson's disease.
Parkinson Disease
Ubiquinone (coenzyme Q10) and complex I in mitochondrial oxidative disorder of Parkinson's disease.
Parkinson Disease
Ubiquinone (coenzyme q10) and mitochondria in oxidative stress of parkinson's disease.
Parkinson Disease
Ubiquinone-quantum dot bioconjugates for in vitro and intracellular complex I sensing.
Parkinson Disease
Unaltered aconitase activity, but decreased complex I activity in substantia nigra pars compacta of patients with Parkinson's disease.
Parkinson Disease
Unexpected improvements of spatial learning and memory abilities in chronic rotenone intoxicated mice.
Parkinson Disease
Up-regulation of gamma-glutamyl transpeptidase activity following glutathione depletion has a compensatory rather than an inhibitory effect on mitochondrial complex I activity: implications for Parkinson's disease.
Parkinson Disease
Variable effects of chronic subcutaneous administration of rotenone on striatal histology.
Parkinson Disease
[3H]dihydrorotenone binding to NADH: ubiquinone reductase (complex I) of the electron transport chain: an autoradiographic study.
Parkinson Disease
[Etiology and pathogenesis of Parkinson's disease: from mitochondrial dysfunctions to familial Parkinson's disease]
Parkinsonian Disorders
A novel de novo STXBP1 mutation is associated with mitochondrial complex I deficiency and late-onset juvenile-onset parkinsonism.
Parkinsonian Disorders
A novel screen for nuclear mitochondrial gene associations with Parkinson's disease.
Parkinsonian Disorders
Anaerobic glycolysis protection against 1-methy-4-phenylpyridinium (MPP+) toxicity in C6 glioma cells.
Parkinsonian Disorders
Anatomic and disease specificity of NADH CoQ1 reductase (complex I) deficiency in Parkinson's disease.
Parkinsonian Disorders
Annonacin, a lipophilic inhibitor of mitochondrial complex I, induces nigral and striatal neurodegeneration in rats: possible relevance for atypical parkinsonism in Guadeloupe.
Parkinsonian Disorders
Astrocytes protect MN9D neuronal cells against rotenone-induced oxidative stress by a glutathione-dependent mechanism.
Parkinsonian Disorders
Atypical parkinsonism in Guadeloupe: a common risk factor for two closely related phenotypes?
Parkinsonian Disorders
Atypical parkinsonism in the Caribbean island of Guadeloupe: etiological role of the mitochondrial complex I inhibitor annonacin.
Parkinsonian Disorders
Atypical unclassifiable parkinsonism on Guadeloupe: an environmental toxic hypothesis.
Parkinsonian Disorders
Balance is the challenge - The impact of mitochondrial dynamics in Parkinson's disease.
Parkinsonian Disorders
Chronic systemic complex I inhibition induces a hypokinetic multisystem degeneration in rats.
Parkinsonian Disorders
Circadian dysfunction in a rotenone-induced parkinsonian rodent model.
Parkinsonian Disorders
Convergent roles of alpha-synuclein, DA metabolism, and the ubiquitin-proteasome system in nigrostriatal toxicity.
Parkinsonian Disorders
Cyclosporin inhibition of apoptosis induced by mitochondrial complex I toxins.
Parkinsonian Disorders
Deficiencies of NADH and succinate dehydrogenases in degenerative diseases and myopathies.
Parkinsonian Disorders
Distinct role for microglia in rotenone-induced degeneration of dopaminergic neurons.
Parkinsonian Disorders
Dopamine- and cAMP-regulated Phosphoprotein of 32-kDa (DARPP-32)-dependent Activation of Extracellular Signal-regulated Kinase (ERK) and Mammalian Target of Rapamycin Complex 1 (mTORC1) Signaling in Experimental Parkinsonism.
Parkinsonian Disorders
Effects of cannabinoids ?(9)-tetrahydrocannabinol, ?(9)-tetrahydrocannabinolic acid and cannabidiol in MPP+ affected murine mesencephalic cultures.
Parkinsonian Disorders
Electrophysiology and pharmacology of striatal neuronal dysfunction induced by mitochondrial complex I inhibition.
Parkinsonian Disorders
Fermentation and alternative respiration compensate for NADH dehydrogenase deficiency in a prokaryotic model of DJ-1-associated Parkinsonism.
Parkinsonian Disorders
GSK3beta and endoplasmic reticulum stress mediate rotenone-induced death of SK-N-MC neuroblastoma cells.
Parkinsonian Disorders
Guadeloupean parkinsonism: a cluster of progressive supranuclear palsy-like tauopathy.
Parkinsonian Disorders
In a flurry of PINK, mitochondrial bioenergetics takes a leading role in Parkinson's disease.
Parkinsonian Disorders
Inhibition of mitochondrial NADH dehydrogenase by pyridine derivatives and its possible relation to experimental and idiopathic parkinsonism.
Parkinsonian Disorders
Inhibition of mTOR signaling in Parkinson's disease prevents L-DOPA-induced dyskinesia.
Parkinsonian Disorders
Interaction of alpha-phenyl-N-tert-butyl nitrone and alternative electron acceptors with complex I indicates a substrate reduction site upstream from the rotenone binding site.
Parkinsonian Disorders
Intervention of mitochondrial dysfunction-oxidative stress-dependent apoptosis as a possible neuroprotective mechanism of ?-lipoic acid against rotenone-induced parkinsonism and L-dopa toxicity.
Parkinsonian Disorders
Involvement of glyceraldehyde-3-phosphate dehydrogenase in rotenone-induced cell apoptosis: relevance to protein misfolding and aggregation.
Parkinsonian Disorders
Iron- and manganese-catalyzed autoxidation of dopamine in the presence of L-cysteine: possible insights into iron- and manganese-mediated dopaminergic neurotoxicity.
Parkinsonian Disorders
L-dihydroxyphenylalanine and complex I deficiency in Parkinson's disease brain.
Parkinsonian Disorders
Low platelet mitochondrial complex I and complex II/III activity in early untreated Parkinson's disease.
Parkinsonian Disorders
Mechanism of the neuroprotective role of coenzyme Q10 with or without L-dopa in rotenone-induced parkinsonism.
Parkinsonian Disorders
Melatonin protects against 6-OHDA-induced neurotoxicity in rats: a role for mitochondrial complex I activity.
Parkinsonian Disorders
Mesencephalic complex I deficiency does not correlate with parkinsonism in mitochondrial DNA maintenance disorders.
Parkinsonian Disorders
Mitochondrial DNA and primary mitochondrial dysfunction in Parkinson's disease.
Parkinsonian Disorders
Mitochondrial DNA mutations in complex I and tRNA genes in Parkinson's disease.
Parkinsonian Disorders
Mitochondrial NADH dehydrogenase and CYP2D6 genotypes in Lewy-body parkinsonism.
Parkinsonian Disorders
MPP(+) -dependent inhibition of I(h) reduces spontaneous activity and enhances EPSP summation in nigral dopamine neurons.
Parkinsonian Disorders
Neuroleptic medications inhibit complex I of the electron transport chain.
Parkinsonian Disorders
Obligatory role for complex I inhibition in the dopaminergic neurotoxicity of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP).
Parkinsonian Disorders
Pathogenetic mechanisms in hereditary dysfunctions of complex I of the respiratory chain in neurological diseases.
Parkinsonian Disorders
Quantification of acetogenins in Annona muricata linked to atypical parkinsonism in guadeloupe.
Parkinsonian Disorders
Role of mitochondrial dysfunction in Parkinson's disease: Implications for treatment.
Parkinsonian Disorders
Rotenone induces degeneration of photoreceptors and impairs the dopaminergic system in the rat retina.
Parkinsonian Disorders
Synergistic anti-Parkinsonism activity of high doses of B vitamins in a chronic cellular model.
Parkinsonian Disorders
Synphilin-1 exhibits trophic and protective effects against Rotenone toxicity.
Parkinsonian Disorders
Systemic administration of rotenone produces selective damage in the striatum and globus pallidus, but not in the substantia nigra.
Parkinsonian Disorders
The D-loop structure of human mtDNA is destabilized directly by 1-methyl-4-phenylpyridinium ion (MPP+), a parkinsonism-causing toxin.
Parkinsonian Disorders
The mitochondrial complex I inhibitor annonacin is toxic to mesencephalic dopaminergic neurons by impairment of energy metabolism.
Parkinsonian Disorders
Trichloroethylene: Parkinsonism and complex 1 mitochondrial neurotoxicity.
Parkinsonian Disorders
Unveiling the role of the pesticides paraquat and rotenone on ?-synuclein fibrillation in vitro.
Parkinsonian Disorders
[Cognitive impairment and parkinsonism due to use of sodium valproate]
Pelizaeus-Merzbacher Disease
The use of targeted genomic capture and massively parallel sequencing in diagnosis of Chinese Leukoencephalopathies.
Peri-Implantitis
A Fluorescent Zn(II)-Coordination Polymer: Selective Detection of Nitrofurantoin and Prevention of Peri-Implantitis after Ultrafine-Grained Titanium Implant by Reducing Inflammatory Cytokines Release.
Periodontitis
Mitochondrial dysfunction is involved in the aggravation of periodontitis by diabetes.
Peripheral Arterial Disease
Decreased NADH dehydrogenase and ubiquinol-cytochrome c oxidoreductase in peripheral arterial disease.
Peripheral Arterial Disease
Mitochondrial complex I defect resulting from exercise-induced lower limb ischemia in patients with peripheral arterial disease.
Peripheral Nerve Injuries
Coupling of serotonergic input to NMDA receptor-phosphorylation following peripheral nerve injury via rapid, synaptic up-regulation of ND2.
Peripheral Nervous System Diseases
A disease-associated Aifm1 variant induces severe myopathy in knockin mice.
Peripheral Nervous System Diseases
A Novel Missense Mutation in AIFM1 Results in Axonal Polyneuropathy and Misassembly of OXPHOS Complexes.
Peripheral Nervous System Diseases
Bioenergetic deficits in peripheral nerve sensory axons during chemotherapy-induced neuropathic pain resulting from peroxynitrite-mediated post-translational nitration of mitochondrial superoxide dismutase.
Peripheral Nervous System Diseases
Familial mitochondrial myopathy associated with peripheral neuropathy: partial deficiencies of complex I and complex IV.
Peripheral Nervous System Diseases
Therapy of complex I deficiency: peripheral neuropathy during dichloroacetate therapy.
Peripheral Nervous System Diseases
[A case of mitochondrial encephalomyopathy with a defect in electron transport at complex I and IV in skeletal muscle showing peripheral neuropathy]
Peripheral Nervous System Diseases
[Multiple symmetric lipomatosis associated to polyneuropathology, atrophy of the cerebellum and mitochondrial cytopathy]
Peripheral Nervous System Diseases
[PROVISIONAL] Whole exome sequencing identifies a novel variant in an apoptosis-inducing factor gene associated with X-linked recessive hearing loss in a Chinese Family.
Peripheral Vascular Diseases
Mitochondrial Respiration after One Session of Calf Raise Exercise in Patients with Peripheral Vascular Disease and Healthy Older Adults.
Perivascular Epithelioid Cell Neoplasms
Benign clear cell "sugar" tumor of the lung in a patient with Birt-Hogg-Dubé syndrome: a case report.
Perivascular Epithelioid Cell Neoplasms
Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa).
Persistent Hyperplastic Primary Vitreous
Modulating EGFR-MTORC1-autophagy as a potential therapy for persistent fetal vasculature (PFV) disease.
Peste-des-Petits-Ruminants
Autophagy in farm animals: current knowledge and future challenges.
Phenylketonurias
Assessment of mitochondrial respiratory chain function in hyperphenylalaninaemia.
Pheochromocytoma
1-Methyl-4-(2'-ethylphenyl)-1,2,3,6-tetrahydropyridine-induced toxicity in PC12 cells is enhanced by preventing glycolysis.
Pheochromocytoma
NADPH Oxidase 2 Mediates Intermittent Hypoxia-Induced Mitochondrial Complex I Inhibition: Relevance to Blood Pressure Changes in Rats.
Pheochromocytoma
Purine nucleoside-mediated protection of chemical hypoxia-induced neuronal injuries involves p42/44 MAPK activation.
Photophobia
Bio-inspired, side-on attachment of a ruthenium photosensitizer to an iron hydrogenase active site model.
Photosensitivity Disorders
Mechanisms of mitochondrial photosensitization by the cationic dye, N,N-bis(2-ethyl-1,3-dioxylene)kryptocyanine (EDKC): preferential inactivation of complex I in the electron transport chain.
Photosensitivity Disorders
Phthalocyanine-photosensitized inactivation of a pathogenic protozoan, Acanthamoeba palestinensis.
Pituitary Neoplasms
Identification of the potential therapeutic target gene UBE2C in human hepatocellular carcinoma: An investigation based on GEO and TCGA databases.
Pituitary Neoplasms
Somatic complex I disruptive mitochondrial DNA mutations are modifiers of tumorigenesis that correlate with low genomic instability in pituitary adenomas.
Pneumonia
Oxidative stress-induced mitochondrial dysfunction drives inflammation and airway smooth muscle remodeling in patients with chronic obstructive pulmonary disease.
Pneumothorax
Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa).
Polycystic Kidney Diseases
An mTOR kinase inhibitor slows disease progression in a rat model of polycystic kidney disease.
Polycystic Kidney Diseases
Effects of TORC1 Inhibition during the Early and Established Phases of Polycystic Kidney Disease.
Polycystic Kidney Diseases
PKD2/polycystin-2 induces autophagy by forming a complex with BECN1.
Polycystic Kidney, Autosomal Dominant
An mTOR anti-sense oligonucleotide decreases polycystic kidney disease in mice with a targeted mutation in Pkd2.
Polycystic Kidney, Autosomal Dominant
PKD2/polycystin-2 induces autophagy by forming a complex with BECN1.
Polycystic Kidney, Autosomal Dominant
Targeting AMP-activated protein kinase (AMPK) for treatment of autosomal dominant polycystic kidney disease.
Polycystic Ovary Syndrome
Mitochondrial complex I impairment in leukocytes from polycystic ovary syndrome patients with insulin resistance.
Polyneuropathies
A newly distal hereditary motor neuropathy caused by a rare AIFM1 mutation.
Polyneuropathies
A Novel Missense Mutation in AIFM1 Results in Axonal Polyneuropathy and Misassembly of OXPHOS Complexes.
Postural Orthostatic Tachycardia Syndrome
A screen of candidate genes and influence of beta2-adrenergic receptor genotypes in postural tachycardia syndrome.
Pre-Eclampsia
Diagnostic Significance of Phosphoinositide 3-Kinase and Mammalian Target of Rapamycin Complex 1 in Preeclampsia.
Pre-Eclampsia
Effect of Preeclampsia on Placental Function: Influence of Sexual Dimorphism, microRNA's and Mitochondria.
Pre-Eclampsia
Evidence for lysosomal biogenesis proteome defect and impaired autophagy in preeclampsia.
Precursor Cell Lymphoblastic Leukemia-Lymphoma
AMP-dependent kinase/mammalian target of rapamycin complex 1 signaling in T-cell acute lymphoblastic leukemia: therapeutic implications.
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Loss of mTOR complex 1 induces developmental blockage in early T-lymphopoiesis and eradicates T-cell acute lymphoblastic leukemia cells.
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Metabolic determinants of cellular fitness dependent on mitochondrial reactive oxygen species.
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma
AMPK Is Essential to Balance Glycolysis and Mitochondrial Metabolism to Control T-ALL Cell Stress and Survival.
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma
Control of amino acid transport coordinates metabolic reprogramming in T-cell malignancy.
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma
MNK1 inhibitor CGP57380 overcomes mTOR inhibitor-induced activation of eIF4E: the mechanism of synergic killing of human T-ALL cells.
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma
Reprogramming of leukemic cell metabolism through the naphthoquinonic compound Quambalarine B.
Premature Birth
Heightened uterine mammalian target of rapamycin complex 1 (mTORC1) signaling provokes preterm birth in mice.
Premature Birth
Metformin attenuates susceptibility to inflammation-induced preterm birth in mice with higher endocannabinoid levels.
Prostatic Neoplasms
A Phase II Trial of Temsirolimus in Men With Castration-Resistant Metastatic Prostate Cancer.
Prostatic Neoplasms
A Positive Step toward Understanding Double-Negative Metastatic Prostate Cancer.
Prostatic Neoplasms
Aberrant Rheb-mediated mTORC1 activation and Pten haploinsufficiency are cooperative oncogenic events.
Prostatic Neoplasms
Ago-RIP-Seq identifies Polycomb repressive complex I member CBX7 as a major target of miR-375 in prostate cancer progression.
Prostatic Neoplasms
An anti-prostate cancer benzofuran-conjugated iridium(III) complex as a dual inhibitor of STAT3 and NF-?B.
Prostatic Neoplasms
An inverted CAV1 (caveolin 1) topology defines novel autophagy-dependent exosome secretion from prostate cancer cells.
Prostatic Neoplasms
Bioenergetic and Antiapoptotic Properties of Mitochondria from Cultured Human Prostate Cancer Cell Lines PC-3, DU145 and LNCaP.
Prostatic Neoplasms
BMI1 regulates androgen receptor in prostate cancer independently of the polycomb repressive complex 1.
Prostatic Neoplasms
Concurrent HDAC and mTORC1 inhibition attenuate androgen receptor and hypoxia signaling associated with alterations in microRNA expression.
Prostatic Neoplasms
Effects of plasmid-based Stat3-specific short hairpin RNA and GRIM-19 on PC-3M tumor cell growth.
Prostatic Neoplasms
Glutamine Transporters Are Targets of Multiple Oncogenic Signaling Pathways in Prostate Cancer.
Prostatic Neoplasms
Highly Promising Antitumor Agent of a Novel Platinum(II) Complex Bearing a Tetradentate Chelating Ligand.
Prostatic Neoplasms
Hydroxylated Rotenoids Selectively Inhibit the Proliferation of Prostate Cancer Cells.
Prostatic Neoplasms
Identifying Mitochondrial-Related Genes NDUFA10 and NDUFV2 as Prognostic Markers for Prostate Cancer through Biclustering.
Prostatic Neoplasms
Inhibition of miR-423-5p suppressed prostate cancer through targeting GRIM-19.
Prostatic Neoplasms
Mitochondrial mutations in early stage prostate cancer and bodily fluids.
Prostatic Neoplasms
Molecular interplay of the noncoding RNA ANRIL and methylated histone H3 lysine 27 by polycomb CBX7 in transcriptional silencing of INK4a.
Prostatic Neoplasms
Plasmid-based Survivin shRNA and GRIM-19 carried by attenuated Salmonella suppresses tumor cell growth.
Prostatic Neoplasms
Re: The Polycomb Repressor Complex 1 Drives Double-Negative Prostate Cancer Metastasis by Coordinating Stemness and Immune Suppression.
Prostatic Neoplasms
Synthesis, oxidant properties, and antitumoral effects of a heteroleptic palladium(II) complex of curcumin on human prostate cancer cells.
Prostatic Neoplasms
The Polycomb Repressor Complex 1 Drives Double-Negative Prostate Cancer Metastasis by Coordinating Stemness and Immune Suppression.
Prostatic Neoplasms
Tuberous Sclerosis Complex 1: An Epithelial Tumor Suppressor Essential to Prevent Spontaneous Prostate Cancer in Aged Mice.
Prostatic Neoplasms
Upregulation of GRIM-19 augments the sensitivity of prostate cancer cells to docetaxel by targeting Rad23b.
protein acetyllysine n-acetyltransferase deficiency
Sirtuin 3 deficiency accelerates Angiotensin II-induced skeletal muscle atrophy.
Protein Deficiency
Protein malnutrition prevents heat conservation induced by amino acid infusion during general anesthesia in rats.
Protein Deficiency
[State of the energy-supply system of the liver mitochondria under the conditions of alimentary deficiency of protein].
Proteinuria
Mitochondrial Complex I Deficiency among Egyptian Pediatric Patients with Steroid-Resistant Nephrotic Syndrome.
Proteinuria
mTORC1 activation triggers the unfolded protein response in podocytes and leads to nephrotic syndrome.
Psoriasis
Lysosome Alterations in the Human Epithelial Cell Line HaCaT and Skin Specimens: Relevance to Psoriasis.
Psoriasis
Mechanistic target of rapamycin complex 1 is involved in psoriasis and regulated by anti-TNF-? treatment.
Psoriasis
Natural Loss-of-Function Mutations in Qa2 and NCF1 Cause the Spread of Mannan-Induced Psoriasis.
Psoriasis
Sustained improvement of psoriatic lesions in the course of sublingual immunotherapy for airborne allergens: clinical evidence of cross-tolerance.
Pulmonary Arterial Hypertension
Up-regulation of the mammalian target of rapamycin complex 1 subunit Raptor by aldosterone induces abnormal pulmonary artery smooth muscle cell survival patterns to promote pulmonary arterial hypertension.
Pulmonary Disease, Chronic Obstructive
Altered mitochondrial regulation in quadriceps muscles of patients with COPD.
Pulmonary Disease, Chronic Obstructive
Characterization and X-ray structure of the NADH-dependent coenzyme A disulfide reductase from Thermus thermophilus.
Pulmonary Disease, Chronic Obstructive
Quadriceps exercise intolerance in patients with chronic obstructive pulmonary disease: the potential role of altered skeletal muscle mitochondrial respiration.
Pulmonary Disease, Chronic Obstructive
Role of long non-coding RNA MALAT1 in chronic obstructive pulmonary disease.
pyruvate dehydrogenase (nadp+) deficiency
Ultrastructural examination of skin biopsies may assist in diagnosing mitochondrial cytopathy when muscle biopsies yield negative results.
Pyruvate Dehydrogenase Complex Deficiency Disease
A Diagnostic Algorithm for Mitochondrial Disorders in Estonian Children.
Pyruvate Dehydrogenase Complex Deficiency Disease
High resolution respirometry of permeabilized skeletal muscle fibers in the diagnosis of neuromuscular disorders.
Pyruvate Dehydrogenase Complex Deficiency Disease
The expanding clinical spectrum of mitochondrial diseases.
Pyruvate Dehydrogenase Complex Deficiency Disease
Ultrastructural examination of skin biopsies may assist in diagnosing mitochondrial cytopathy when muscle biopsies yield negative results.
pyruvate dehydrogenase system deficiency
Neonatal onset of mitochondrial disorders in 129 patients: clinical and laboratory characteristics and a new approach to diagnosis.
Rabies
Rabies virus phosphoprotein interacts with mitochondrial Complex I and induces mitochondrial dysfunction and oxidative stress.
Reflex, Abnormal
[Multiple symmetric lipomatosis associated to polyneuropathology, atrophy of the cerebellum and mitochondrial cytopathy]
Renal Insufficiency
A novel mitochondrial MTND5 frameshift mutation causing isolated complex I deficiency, renal failure and myopathy.
Renal Insufficiency
Biallelic Mutations in TMEM126B Cause Severe Complex I Deficiency with a Variable Clinical Phenotype.
Renal Insufficiency
Inpp5e suppresses polycystic kidney disease via inhibition of PI3K/Akt-dependent mTORC1 signaling.
Renal Insufficiency, Chronic
Canagliflozin mediated dual inhibition of mitochondrial glutamate dehydrogenase and complex I: an off-target adverse effect.
Renal Insufficiency, Chronic
Deficiency in Mitochondrial Complex I Activity Due to Ndufs6 Gene Trap Insertion Induces Renal Disease.
Renal Insufficiency, Chronic
Podocyte-specific deletion of tubular sclerosis complex 2 promotes focal segmental glomerulosclerosis and progressive renal failure.
Reperfusion Injury
3, 4-dihydroxyl-phenyl lactic acid restores NADH dehydrogenase 1 ? subunit 10 to ameliorate cardiac reperfusion injury.
Reperfusion Injury
A mitochondria-targeted S-nitrosothiol modulates respiration, nitrosates thiols, and protects against ischemia-reperfusion injury.
Reperfusion Injury
Cardioprotection by S-nitrosation of a cysteine switch on mitochondrial complex I.
Reperfusion Injury
Central role for aldose reductase pathway in myocardial ischemic injury.
Reperfusion Injury
Control of mitochondrial superoxide production by reverse electron transport at complex I.
Reperfusion Injury
Cryo-EM structures of complex I from mouse heart mitochondria in two biochemically defined states.
Reperfusion Injury
Damage to mitochondrial complex I during cardiac ischemia reperfusion injury is reduced indirectly by anti-anginal drug ranolazine.
Reperfusion Injury
Eicosapentaenoic acid attenuates renal lipotoxicity by restoring autophagic flux.
Reperfusion Injury
Electron leak from NDUFA13 within mitochondrial complex I attenuates ischemia-reperfusion injury via dimerized STAT3.
Reperfusion Injury
Ischemic A/D transition of mitochondrial complex I and its role in ROS generation.
Reperfusion Injury
Mitochondrial Complex I Inhibition by Metformin Limits Reperfusion Injury.
Reperfusion Injury
Mitochondrial dysfunction in cardiac ischemia-reperfusion injury: ROS from complex I, without inhibition.
Reperfusion Injury
Mitochondrial respiratory chain complexes as sources and targets of thiol-based redox-regulation.
Reperfusion Injury
Nitric oxide attenuates cardiomyocytic apoptosis via diminished mitochondrial complex I up-regulation from cardiac ischemia-reperfusion injury under cardiopulmonary bypass.
Reperfusion Injury
Partial loss of complex I due to NDUFS4 deficiency augments myocardial reperfusion damage by increasing mitochondrial superoxide/hydrogen peroxide production.
Reperfusion Injury
Protective effect of melatonin against mitochondrial dysfunction associated with cardiac ischemia- reperfusion: role of cardiolipin.
Reperfusion Injury
Reactive oxygen species production induced by pore opening in cardiac mitochondria: The role of complex III.
Reperfusion Injury
Reverse electron transfer results in a loss of flavin from mitochondrial complex I: Potential mechanism for brain ischemia reperfusion injury.
Respiratory Insufficiency
A recurring mutation in the respiratory complex 1 protein NDUFB11 is responsible for a novel form of X-linked sideroblastic anemia.
Respiratory Insufficiency
Characterization of oxygen-resistant Chinese hamster ovary cells. III. Relative resistance of succinate and alpha-ketoglutarate dehydrogenases to hyperoxic inactivation.
Respiratory Insufficiency
Clinical presentation of mitochondrial respiratory chain defects in NADH-coenzyme Q reductase and cytochrome oxidase: clues to pathogenesis of Leigh disease.
Respiratory Insufficiency
Fatal neonatal lactic acidosis with respiratory insufficiency due to complex I and IV deficiency.
Respiratory Insufficiency
Human, Tissue-Engineered, Skeletal Muscle Myobundles to Measure Oxygen Uptake and Assess Mitochondrial Toxicity.
Respiratory Insufficiency
Inhibition of complex I by isoquinoline derivatives structurally related to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP).
Respiratory Insufficiency
Isoflurane anesthetic hypersensitivity and progressive respiratory depression in a mouse model with isolated mitochondrial complex I deficiency.
Respiratory Tract Infections
In vitro and murine efficacy and toxicity studies of nebulized SCC1, a methylated caffeine-silver(I) complex, for treatment of pulmonary infections.
Retinal Degeneration
Apoptosis inducing factor deficiency causes retinal photoreceptor degeneration. The protective role of the redox compound methylene blue.
Retinal Degeneration
Rotenone-induced inner retinal degeneration via presynaptic activation of voltage-dependent sodium and L-type calcium channels in rats.
Retinitis Pigmentosa
A novel mutation in the mitochondrial tRNA(Pro) gene associated with late-onset ataxia, retinitis pigmentosa, deafness, leukoencephalopathy and complex I deficiency.
Retinoblastoma
Prognostic significance of mitochondrial oxidative phosphorylation complexes: Therapeutic target in the treatment of retinoblastoma.
Retinoblastoma
The expression of mitochondrial complexes I and III correlates with prognosis or diagnosis in retinoblastoma: immunohistochemical and histopathological parameters.
Retinoblastoma
Thoc1 inhibits cell growth via induction of cell cycle arrest and apoptosis in lung cancer cells.
Retinoblastoma
Tumor suppressor SMAR1 mediates cyclin D1 repression by recruitment of the SIN3/histone deacetylase 1 complex.
Rett Syndrome
Dysregulation of Mammalian Target of Rapamycin Signaling in Mouse Models of Autism.
Reye Syndrome
Neonatal multiorgan failure due to ACAD9 mutation and complex I deficiency with mitochondrial hyperplasia in liver, cardiac myocytes, skeletal muscle, and renal tubules.
Reye Syndrome
Resveratrol protects against experimental induced Reye's syndrome by prohibition of oxidative stress and restoration of complex I activity.
Rhabdomyolysis
Hemolytic uremic syndrome and rhabdomyolysis in a patient with succinate coenzyme Q reductase (complex II) deficiency.
Rhinitis, Allergic
Sustained improvement of psoriatic lesions in the course of sublingual immunotherapy for airborne allergens: clinical evidence of cross-tolerance.
Rickets, Hypophosphatemic
Successful Medical Therapy for Hypophosphatemic Rickets due to Mitochondrial Complex I Deficiency Induced de Toni-Debré-Fanconi Syndrome.
Root Resorption
Microcracks on the Rat Root Surface Induced by Orthodontic Force, Crack Extension Simulation, and Proteomics Study.
Sarcoidosis
High expression of mTOR signaling in granulomatous lesions is not predictive for the clinical course of sarcoidosis.
Sarcoma
Bioenergetic properties of human sarcoma cells help define sensitivity to metabolic inhibitors.
Sarcoma
Differential inhibition/inactivation of mitochondrial complex I implicates its alteration in malignant cells.
Sarcoma
Efficacy of Ganitumab (AMG 479), Alone and in Combination With Rapamycin, in Ewing's and Osteogenic Sarcoma Models.
Sarcoma
Induction of Moloney murine sarcoma virus tolerance in adult mice by anti-CD4 monoclonal antibody treatment.
Sarcoma
Mutually exclusive BCOR internal tandem duplications and YWHAE-NUTM2 fusions in clear cell sarcoma of kidney: not the full story.
Sarcoma
Role of the MAPK/cJun NH2-terminal kinase signaling pathway in starvation-induced autophagy.
Sarcoma, Clear Cell
Mutually exclusive BCOR internal tandem duplications and YWHAE-NUTM2 fusions in clear cell sarcoma of kidney: not the full story.
Sarcoma, Kaposi
Kaposi sarcoma-associated herpesvirus miRNAs suppress CASTOR1-mediated mTORC1 inhibition to promote tumorigenesis.
Sarcopenia
Naked mole-rats maintain healthy skeletal muscle and Complex IV mitochondrial enzyme function into old age.
Sarcopenia
Sarcopenia, age, atrophy, and myopathy: Mitochondrial oxidative enzyme activities.
Seizures
Aplasia of the retinal vessels combined with optic nerve hypoplasia, neonatal epileptic seizures, and lactic acidosis due to mitochondrial complex I deficiency.
Seizures
Contribution of nitric oxide, superoxide anion, and peroxynitrite to activation of mitochondrial apoptotic signaling in hippocampal CA3 subfield following experimental temporal lobe status epilepticus.
Seizures
Depletion of reduced glutathione precedes inactivation of mitochondrial enzymes following limbic status epilepticus in the rat hippocampus.
Seizures
Facilitation of seizures in limbic epileptic rats by complex 1 microTesla magnetic fields.
Seizures
In Vivo AAV1 Transduction With hRheb(S16H) Protects Hippocampal Neurons by BDNF Production.
Seizures
Mammalian target of rapamycin complex 1 activation negatively regulates Polo-like kinase 2-mediated homeostatic compensation following neonatal seizures.
Seizures
Mitochondrial complex I inhibition in cerebral cortex of immature rats following homocysteic acid-induced seizures.
Seizures
Mitochondrial dysfunction and ultrastructural damage in the hippocampus during kainic acid-induced status epilepticus in the rat.
Seizures
Morin Prevents Granule Cell Dispersion and Neurotoxicity via Suppression of mTORC1 in a Kainic Acid-induced Seizure Model.
Seizures
Naringenin ameliorates kainic acid-induced morphological alterations in the dentate gyrus in a mouse model of temporal lobe epilepsy.
Seizures
Postnatal reduction of tuberous sclerosis complex 1 expression in astrocytes and neurons causes seizures in an age-dependent manner.
Seizures
Recombinant human erythropoietin protects against brain injury through blunting the mTORC1 pathway in the developing brains of rats with seizures.
Seizures
Seizure-dependent modulation of mitochondrial oxidative phosphorylation in rat hippocampus.
Seizures
Selective inhibition of mTORC1/2 or PI3K/mTORC1/2 signaling does not prevent or modify epilepsy in the intrahippocampal kainate mouse model.
Seizures
Sirolimus for epilepsy in children with tuberous sclerosis complex: A randomized controlled trial.
Seizures
Sustained deficiency of mitochondrial complex I activity during long periods of survival after seizures induced in immature rats by homocysteic acid.
Seizures
Targeting deficiencies in mitochondrial respiratory complex I and functional uncoupling exerts anti-seizure effects in a genetic model of temporal lobe epilepsy and in a model of acute temporal lobe seizures.
Seizures
The interaction between early life epilepsy and autistic-like behavioral consequences: a role for the mammalian target of rapamycin (mTOR) pathway.
Seizures
Treatment With Resveratrol Ameliorates Mitochondrial Dysfunction During the Acute Phase of Status Epilepticus in Immature Rats.
Seminoma
Synthesis of a novel platinum(II) complex with 6,7-dichloro-5,8-quinolinedione and the study of its antitumor mechanism in testicular seminoma.
Sepsis
Alterations in Mitochondrial Function in Blood Cells Obtained from Patients with Sepsis Presenting to an Emergency Department.
Sepsis
Differential Regulation of the Autophagy and Proteasome Pathways in Skeletal Muscles in Sepsis.
Sepsis
Impaired energy metabolism in hearts of septic baboons: diminished activities of Complex I and Complex II of the mitochondrial respiratory chain.
Sepsis
Interaction of alpha-phenyl-N-tert-butyl nitrone and alternative electron acceptors with complex I indicates a substrate reduction site upstream from the rotenone binding site.
Sepsis
MiR-210-3p Enhances Cardiomyocyte Apoptosis and Mitochondrial Dysfunction by Targeting the NDUFA4 Gene in Sepsis-Induced Myocardial Dysfunction.
Sepsis
Persistent S-nitrosation of complex I and other mitochondrial membrane proteins by S-nitrosothiols but not nitric oxide or peroxynitrite: implications for the interaction of nitric oxide with mitochondria.
Sepsis
Protective effects of hydrogen gas against sepsis-induced acute lung injury via regulation of mitochondrial function and dynamics.
Sepsis
Recombinant human mitochondrial transcription factor A stimulates mitochondrial biogenesis and ATP synthesis, improves motor function after MPTP, reduces oxidative stress and increases survival after endotoxin.
serine c-palmitoyltransferase deficiency
Loss of Neurological Disease HSAN-I-Associated Gene SPTLC2 Impairs CD8+ T Cell Responses to Infection by Inhibiting T Cell Metabolic Fitness.
Shock, Cardiogenic
Suppression of Superoxide-Hydrogen Peroxide Production at Site IQ of Mitochondrial Complex I Attenuates Myocardial Stunning and Improves Postcardiac Arrest Outcomes.
Shock, Septic
Blood Levels of Free-Circulating Mitochondrial DNA in Septic Shock and Postsurgical Systemic Inflammation and Its Influence on Coagulation: A Secondary Analysis of a Prospective Observational Study.
Shock, Septic
Impaired energy metabolism in hearts of septic baboons: diminished activities of Complex I and Complex II of the mitochondrial respiratory chain.
Shock, Septic
Inhibition of mitochondrial respiratory complex I by nitric oxide, peroxynitrite and S-nitrosothiols.
Shock, Septic
Mitochondrial changes in platelets are not related to those in skeletal muscle during human septic shock.
Shock, Septic
Mitochondrial dysfunction in sepsis: evidence from bacteraemic baboons and endotoxaemic rabbits.
Skin Neoplasms
Negative regulation of the FOXO3a transcription factor by mTORC2 induces a pro-survival response following exposure to ultraviolet-B irradiation.
Smith-Magenis Syndrome
C9orf72 and smcr8 mutant mice reveal MTORC1 activation due to impaired lysosomal degradation and exocytosis.
Spasms, Infantile
Aplasia of the retinal vessels combined with optic nerve hypoplasia, neonatal epileptic seizures, and lactic acidosis due to mitochondrial complex I deficiency.
Spasms, Infantile
Isolated cortical tuber in an infant with genetically confirmed tuberous sclerosis complex 1 presenting with symptomatic West syndrome.
Spasms, Infantile
Respiratory chain complex I deficiency in an infant with infantile spasms.
Spastic Paraplegia, Hereditary
Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia.
Spinal Cord Diseases
Myelopathy in Two Brothers with Respiratory Chain Disorder-Severe Complex 1 Deficiency with Atlantoaxial Dislocation and Long Spinal Arachnoid Cyst: A New Unreported Association.
Spinal Cord Diseases
Neurological complications of HIV-1-seropositive internal medicine inpatients in Kinshasa, Zaire.
Spinal Cord Injuries
Inhibition of mammalian target of rapamycin complex 1 signaling by n-3 polyunsaturated fatty acids promotes locomotor recovery after spinal cord injury.
Spinocerebellar Ataxias
Infantile-onset spinocerebellar ataxia and mitochondrial recessive ataxia syndrome are associated with neuronal complex I defect and mtDNA depletion.
Spinocerebellar Ataxias
Mitochondrial complex I gene variant associated with early age at onset in spinocerebellar ataxia type 2.
Spinocerebellar Ataxias
SETX (senataxin), the helicase mutated in AOA2 and ALS4, functions in autophagy regulation.
Spinocerebellar Ataxias
Short-term succinic acid treatment mitigates cerebellar mitochondrial OXPHOS dysfunction, neurodegeneration and ataxia in a Purkinje-specific spinocerebellar ataxia type 1 (SCA1) mouse model.
Squamous Cell Carcinoma of Head and Neck
A Phase 1 Study of Everolimus + Weekly Cisplatin + Intensity Modulated Radiation Therapy in Head-and-Neck Cancer.
Squamous Cell Carcinoma of Head and Neck
A phase I study of temsirolimus plus carboplatin plus paclitaxel for patients with recurrent or metastatic (R/M) head and neck squamous cell cancer (HNSCC).
Squamous Cell Carcinoma of Head and Neck
Arecoline downregulates levels of p21 and p27 through the reactive oxygen species/mTOR complex 1 pathway and may contribute to oral squamous cell carcinoma.
Squamous Cell Carcinoma of Head and Neck
Chemopreventive and Chemotherapeutic Actions of mTOR Inhibitor in Genetically Defined Head and Neck Squamous Cell Carcinoma Mouse Model.
Squamous Cell Carcinoma of Head and Neck
Decreased expression of GRIM-19 by DNA hypermethylation promotes aerobic glycolysis and cell proliferation in head and neck squamous cell carcinoma.
Squamous Cell Carcinoma of Head and Neck
Differential expression of organic cation transporter OCT-3 in oral premalignant and malignant lesions: potential implications in the antineoplastic effects of metformin.
Squamous Cell Carcinoma of Head and Neck
Metformin Clinical Trial in HPV+ and HPV- Head and Neck Squamous Cell Carcinoma: Impact on Cancer Cell Apoptosis and Immune Infiltrate.
Squamous Cell Carcinoma of Head and Neck
Metformin Inhibits Progression of Head and Neck Squamous Cell Carcinoma by Acting Directly on Carcinoma-Initiating Cells.
Squamous Cell Carcinoma of Head and Neck
Metformin prevents the development of oral squamous cell carcinomas from carcinogen-induced premalignant lesions.
Squamous Cell Carcinoma of Head and Neck
Upregulation of GRIM-19 suppresses the growth of oral squamous cell carcinoma in vitro and in vivo.
Starvation
A missing piece of the puzzle: Atg11 functions as a scaffold to activate Atg1 for selective autophagy.
Starvation
A novel mechanism for the control of translation initiation by amino acids, mediated by phosphorylation of eukaryotic initiation factor 2B.
Starvation
Activation of mTORC1 under nutrient starvation conditions increases cellular radiosensitivity in human liver cancer cell lines, HepG2 and HuH6.
Starvation
Albumin prevents reactive oxygen species-induced mitochondrial damage, autophagy, and apoptosis during serum starvation.
Starvation
Amino acids and autophagy: cross-talk and co-operation to control cellular homeostasis.
Starvation
AMPK-dependent phosphorylation is required for transcriptional activation of TFEB and TFE3.
Starvation
Cdc14 protein phosphatase and topoisomerase II mediate rDNA dynamics and nucleophagic degradation of nucleolar proteins after TORC1 inactivation.
Starvation
CLIP and cohibin separate rDNA from nucleolar proteins destined for degradation by nucleophagy.
Starvation
Divergent targets of glycolysis and oxidative phosphorylation result in additive effects of metformin and starvation in colon and breast cancer.
Starvation
ERK7 is a negative regulator of protein secretion in response to amino-acid starvation by modulating Sec16 membrane association.
Starvation
General Control Nonderepressible 2 (GCN2) Kinase Inhibits Target of Rapamycin Complex 1 in Response to Amino Acid Starvation in
Starvation
Glycolytic reprogramming of macrophages activated by NOD1 and TLR4 agonists: No association with proinflammatory cytokine production in normoxia.
Starvation
Growth-factor dependent expression of the translationally controlled tumour protein TCTP is regulated through the PI3-K/Akt/mTORC1 signalling pathway.
Starvation
H2A Monoubiquitination Links Glucose Availability to Epigenetic Regulation of the Endoplasmic Reticulum Stress Response and Cancer Cell Death.
Starvation
Inactivation of Rheb by PRAK-mediated phosphorylation is essential for energy-depletion-induced suppression of mTORC1.
Starvation
Loss of CLN7 results in depletion of soluble lysosomal proteins and impaired mTOR reactivation.
Starvation
MIR145-3p promotes autophagy and enhances bortezomib sensitivity in multiple myeloma by targeting HDAC4.
Starvation
Mutants defective in the energy-conserving NADH dehydrogenase of Salmonella typhimurium identified by a decrease in energy-dependent proteolysis after carbon starvation.
Starvation
PAT4 levels control amino-acid sensitivity of rapamycin-resistant mTORC1 from the Golgi and affect clinical outcome in colorectal cancer.
Starvation
Rapamycin increases rDNA stability by enhancing association of Sir2 with rDNA in Saccharomyces cerevisiae.
Starvation
rDNA Condensation Promotes rDNA Separation from Nucleolar Proteins Degraded for Nucleophagy after TORC1 Inactivation.
Starvation
Relationship between respiratory enzymes and survival of Escherichia coli under starvation stress in lake water.
Starvation
Rim15 and Sch9 kinases are involved in induction of autophagic degradation of ribosomes in budding yeast.
Starvation
Single amino-acid changes that confer constitutive activation of mTOR are discovered in human cancer.
Starvation
Sorting nexin Mdm1/SNX14 regulates nucleolar dynamics at the NVJ after TORC1 inactivation.
Starvation
Structural pathway for allosteric activation of the autophagic PI 3-kinase complex I.
Starvation
The TOR complex 1 is required for the interaction of multiple cargo proteins selected for the vacuole import and degradation pathway.
Starvation
The TORC1-Nem1/Spo7-Pah1/lipin axis regulates microautophagy induction in budding yeast.
Starvation
TNFAIP8L2/TIPE2 impairs autolysosome reformation via modulating the RAC1-MTORC1 axis.
Starvation
TORC1 Regulates Developmental Responses to Nitrogen Stress via Regulation of the GATA Transcription Factor Gaf1.
Starvation
TORC1 regulates ESCRT-0 complex formation on the vacuolar membrane and microautophagy induction in yeast.
Starvation
TORC1 regulators Iml1/GATOR1 and GATOR2 control meiotic entry and oocyte development in Drosophila.
Starvation
TORC1-dependent sumoylation of Rpc82 promotes RNA polymerase III assembly and activity.
Starvation
Tripartite suppression of fission yeast TORC1 signaling by the GATOR1-Sea3 complex, the TSC complex, and Gcn2 kinase.
Starvation
Vacuole import and degradation pathway: Insights into a specialized autophagy pathway.
Starvation
Yeast DJ-1 superfamily members are required for diauxic-shift reprogramming and cell survival in stationary phase.
Status Epilepticus
Alteration of Gene Associated with Retinoid-interferon-induced Mortality-19-expressing Cell Types in the Mouse Hippocampus Following Pilocarpine-induced Status Epilepticus.
Status Epilepticus
Contribution of nitric oxide, superoxide anion, and peroxynitrite to activation of mitochondrial apoptotic signaling in hippocampal CA3 subfield following experimental temporal lobe status epilepticus.
Status Epilepticus
Mitochondrial dysfunction and oxidative stress in seizure-induced neuronal cell death.
Status Epilepticus
Mitochondrial dysfunction and ultrastructural damage in the hippocampus during kainic acid-induced status epilepticus in the rat.
Stomach Neoplasms
AMPK? phosphatase Ppm1E upregulation in human gastric cancer is required for cell proliferation.
Stomach Neoplasms
Annonaceous acetogenin mimic AA005 induces cancer cell death via apoptosis inducing factor through a caspase-3-independent mechanism.
Stomach Neoplasms
CBX7 regulates stem cell-like properties of gastric cancer cells via p16 and AKT-NF-?B-miR-21 pathways.
Stomach Neoplasms
Deficiency of the complex I of the mitochondrial respiratory chain but improved adenylate control over succinate-dependent respiration are human gastric cancer-specific phenomena.
Stomach Neoplasms
Gastric cancer depends on aldehyde dehydrogenase 3A1 for fatty acid oxidation.
Stomach Neoplasms
Genetic Variants Within MTORC1 Genes Predict Gastric Cancer Prognosis in Chinese Populations.
Stomach Neoplasms
GRIM-19 inhibits the STAT3 signaling pathway and sensitizes gastric cancer cells to radiation.
Stomach Neoplasms
Inhibition of gastric cancer by local anesthetic bupivacaine through multiple mechanisms independent of sodium channel blockade.
Stomach Neoplasms
JS-K induces reactive oxygen species-dependent anti-cancer effects by targeting mitochondria respiratory chain complexes in gastric cancer.
Stomach Neoplasms
Mitochondrial GRIM-19 as a potential therapeutic target for STAT3-dependent carcinogenesis of gastric cancer.
Stomach Neoplasms
Mitochondrial GRIM-19 deficiency facilitates gastric cancer metastasis through oncogenic ROS-NRF2-HO-1 axis via a NRF2-HO-1 loop.
Stomach Neoplasms
Mitochondrial NADH Dehydrogenase Subunit 3 (MTND3) Polymorphisms are Associated with Gastric Cancer Susceptibility.
Stomach Neoplasms
mTORC1 inhibition restricts inflammation-associated gastrointestinal tumorigenesis in mice.
Stomach Neoplasms
NAIF1 inhibits gastric cancer cells migration and invasion via the MAPK pathways.
Stomach Neoplasms
NAIF1 is down-regulated in gastric cancer and promotes apoptosis through the caspase-9 pathway in human MKN45 cells.
Stomach Neoplasms
Overexpression of nuclear apoptosis-inducing factor 1 altered the proteomic profile of human gastric cancer cell MKN45 and induced cell cycle arrest at G1/S phase.
Stomach Neoplasms
Preliminary study on the inhibitory effect of tumor suppressor gene KPC1 on the proliferation in gastric carcinoma cell.
Stomach Neoplasms
Redox state of adipose tissue for patients with gastric cancer and its connection with the body mass index and distance from the tumor.
Stomach Neoplasms
RNA interference targeting raptor inhibits proliferation of gastric cancer cells.
Stomach Neoplasms
TIMMDC1 Knockdown Inhibits Growth and Metastasis of Gastric Cancer Cells through Metabolic Inhibition and AKT/GSK3?/?-Catenin Signaling Pathway.
Stomach Neoplasms
Upregulation of the GRIM-19 gene suppresses invasion and metastasis of human gastric cancer SGC-7901 cell line.
Stroke
Ablation of TMEM126B protects against oxygen-glucose deprivation/reoxygenation-induced injuries of PC12 cells via maintaining mitochondrial anti-apoptotic functions.
Stroke
Critical Role of Flavin and Glutathione in Complex I-Mediated Bioenergetic Failure in Brain Ischemia/Reperfusion Injury.
Stroke
Interaction of alpha-phenyl-N-tert-butyl nitrone and alternative electron acceptors with complex I indicates a substrate reduction site upstream from the rotenone binding site.
Stroke
Mitochondrial complex I deficiency and cardiovascular diseases: current evidence and future directions.
Stroke
Ndufc2 Gene Inhibition Is Associated With Mitochondrial Dysfunction and Increased Stroke Susceptibility in an Animal Model of Complex Human Disease.
Stroke
Pharmacological restoration of autophagy reduces hypertension-related stroke occurrence.
Stroke
Predominant cerebellar volume loss as a neuroradiologic feature of pediatric respiratory chain defects.
Stroke
RapaLink-1 plays an antithrombotic role in antiphospholipid syndrome by improving autophagy both in vivo and vitro.
Stroke
The addition of ketone bodies alleviates mitochondrial dysfunction by restoring complex I assembly in a MELAS cellular model.
Stroke
The reduction of NDUFC2 expression is associated with mitochondrial impairment in circulating mononuclear cells of patients with acute coronary syndrome.
Stroke
Thrombolysis by PLAT/tPA increases serum free IGF1 leading to a decrease of deleterious autophagy following brain ischemia.
Stroke
Very low levels of the mtDNA A3243G mutation associated with mitochondrial dysfunction in vivo.
succinate dehydrogenase deficiency
Analysis of the mitochondrial complex I-v enzyme activities of peripheral leukocytes in oxidative phosphorylation disorders.
succinate dehydrogenase deficiency
Clinical spectrum, morbidity, and mortality in 113 pediatric patients with mitochondrial disease.
succinate dehydrogenase deficiency
Metabolic flexibility of mitochondrial respiratory chain disorders predicted by computer modelling.
succinate dehydrogenase deficiency
Predominant cerebellar volume loss as a neuroradiologic feature of pediatric respiratory chain defects.
succinate dehydrogenase deficiency
SURFEIT-1 gene analysis and two-dimensional blue native gel electrophoresis in cytochrome c oxidase deficiency.
Supranuclear Palsy, Progressive
Electrophysiology and pharmacology of striatal neuronal dysfunction induced by mitochondrial complex I inhibition.
Supranuclear Palsy, Progressive
NR2B-containing NMDA receptors promote the neurotoxic effects of 3-nitropropionic acid but not of rotenone in the striatum.
Supranuclear Palsy, Progressive
Short-term effects of coenzyme Q10 in progressive supranuclear palsy: a randomized, placebo-controlled trial.
Synovitis
Mycobacterial synovitis caused by slow-growing nonchromogenic species: eighteen cases and a review of the literature.
Tachycardia
A screen of candidate genes and influence of beta2-adrenergic receptor genotypes in postural tachycardia syndrome.
Takayasu Arteritis
Targeting the mechanistic target of rapamycin complex 1 restricts pro-inflammatory T cell differentiation and ameliorates Takayasu's arteritis.
Talipes Cavus
[Multiple symmetric lipomatosis associated to polyneuropathology, atrophy of the cerebellum and mitochondrial cytopathy]
Tauopathies
Annonacin, a natural mitochondrial complex I inhibitor, causes tau pathology in cultured neurons.
Tauopathies
Chronic consumption of Annona muricata juice triggers and aggravates cerebral tau phosphorylation in wild-type and MAPT transgenic mice.
Tauopathies
Natural lipophilic inhibitors of mitochondrial complex I are candidate toxins for sporadic neurodegenerative tau pathologies.
Teratocarcinoma
A melatonin-based fluorescence method for the measurement of mitochondrial complex III function in intact cells.
Thiamine Deficiency
A novel mutation in the thiamine responsive megaloblastic anaemia gene SLC19A2 in a patient with deficiency of respiratory chain complex I.
Thrombosis
Lung transcriptome of a COVID-19 patient and systems biology predictions suggest impaired surfactant production which may be druggable by surfactant therapy.
Thymoma
4EBP1/eIF4E and p70S6K/RPS6 axes play critical and distinct roles in hepatocarcinogenesis driven by AKT and N-Ras proto-oncogenes in mice.
Thymoma
A mechanistic target of rapamycin complex 1/2 (mTORC1)/V-Akt murine thymoma viral oncogene homolog 1 (AKT1)/cathepsin H axis controls filaggrin expression and processing in skin, a novel mechanism for skin barrier disruption in patients with atopic dermatitis.
Thymoma
AKT inhibition-mediated dephosphorylation of TFE3 promotes overactive autophagy independent of MTORC1 in cadmium-exposed bone mesenchymal stem cells.
Thymoma
Differential Regulation of the Autophagy and Proteasome Pathways in Skeletal Muscles in Sepsis.
Thymoma
Electroacupuncture ameliorates beta-amyloid pathology and cognitive impairment in Alzheimer disease via a novel mechanism involving activation of TFEB (transcription factor EB).
Thymoma
Mitochondrial respiratory complex I dysfunction promotes tumorigenesis through ROS alteration and AKT activation.
Thymoma
PAQR3 suppresses the growth of non-small cell lung cancer cells via modulation of EGFR-mediated autophagy.
Thymoma
Role of the MAPK/cJun NH2-terminal kinase signaling pathway in starvation-induced autophagy.
Thymoma
Thrombolysis by PLAT/tPA increases serum free IGF1 leading to a decrease of deleterious autophagy following brain ischemia.
Thyroid Cancer, Papillary
Alterations of Oxidative Phosphorylation Complexes in Papillary Thyroid Carcinoma.
Thyroid Cancer, Papillary
Morphoproteomics demonstrates activation of mammalian target of rapamycin pathway in papillary thyroid carcinomas with nuclear translocation of MTOR in aggressive histological variants.
Thyroid Neoplasms
Familial non-medullary thyroid carcinoma: clinico-pathological features, current knowledge and novelty regarding genetic risk factors.
Thyroid Neoplasms
High frequency of somatic mitochondrial DNA mutations in human thyroid carcinomas and complex I respiratory defect in thyroid cancer cell lines.
Thyroid Neoplasms
Non-toxic fragment of botulinum neurotoxin type A and monomethyl auristatin E conjugate for targeted therapy for neuroendocrine tumors.
Thyroid Neoplasms
Plasmid-based Stat3-specific siRNA and GRIM-19 inhibit the growth of thyroid cancer cells in vitro and in vivo.
Thyroid Neoplasms
Point mutation in GRIM-19: a new genetic lesion in Hurthle cell thyroid carcinomas.
Thyroid Neoplasms
The antioxidant function of Bcl-2 preserves cytoskeletal stability of cells with defective respiratory complex I.
Thyroid Neoplasms
Unique landscape of widespread chromosomal losses in Hürthle cell carcinoma.
Toxoplasmosis
Maternal toxoplasmosis and the risk of childhood autism: serological and molecular small-scale studies.
Triple Negative Breast Neoplasms
Activated kinase screening identifies the IKBKE oncogene as a positive regulator of autophagy.
Triple Negative Breast Neoplasms
Selective Inhibition of Lysine-Specific Demethylase 5A (KDM5A) Using a Rhodium(III) Complex for Triple-Negative Breast Cancer Therapy.
Triple Negative Breast Neoplasms
Silver(I) complexes of 3-methoxy-4-hydroxybenzaldehyde thiosemicarbazones and triphenylphosphine: structural, cytotoxicity, and apoptotic studies.
Tuberculosis
2-Mercapto-Quinazolinones as Inhibitors of Type II NADH Dehydrogenase and Mycobacterium tuberculosis: Structure-Activity Relationships, Mechanism of Action and Absorption, Distribution, Metabolism, and Excretion Characterization.
Tuberculosis
Characterization of the Type 2 NADH: Menaquinone oxidoreductases from Staphylococcus aureus and the bactericidal action of phenothiazines.
Tuberculosis
Coupling reporter expression to respiration detects active as well as dormant mycobacteria in vitro and in mouse tissues.
Tuberculosis
Doubly end-on azido bridged mixed-valence cobalt trinuclear complex: Spectral study, VTM, inhibitory effect and antimycobacterial activity on human carcinoma and tuberculosis cells.
Tuberculosis
EPR interpretation, magnetism and biological study of a Cu(II) dinuclear complex assisted by a schiff base precursor.
Tuberculosis
Incorporation of triphenylphosphonium functionality improves the inhibitory properties of phenothiazine derivatives in Mycobacterium tuberculosis.
Tuberculosis
Mode of Action of Clofazimine and Combination Therapy with Benzothiazinones against Mycobacterium tuberculosis.
Tuberculosis
Multiplex PCR assay for the detection of mycobacterial DNA sequences directly from sputum.
Tuberculosis
Mycobacterium tuberculosis type II NADH-menaquinone oxidoreductase catalyzes electron transfer through a two-site ping-pong mechanism and has two quinone-binding sites.
Tuberculosis
Novel mutations in ndh in isoniazid-resistant Mycobacterium tuberculosis isolates.
Tuberculosis
Phenothiazines as anti-tubercular agents: mechanistic insights and clinical implications.
Tuberculosis
Quinolinyl Pyrimidines: Potent Inhibitors of NDH-2 as a Novel Class of Anti-TB Agents.
Tuberculosis
Small Molecules Targeting Mycobacterium tuberculosis Type II NADH Dehydrogenase Exhibit Antimycobacterial Activity.
Tuberculosis
Steady-state kinetics and inhibitory action of antitubercular phenothiazines on mycobacterium tuberculosis type-II NADH-menaquinone oxidoreductase (NDH-2).
Tuberculosis
Stereospecificity of the allosteric NADH dehydrogenase from Mycobacterium tuberculosis.
Tuberculosis
Storage lipid studies in tuberculosis reveal that foam cell biogenesis is disease-specific.
Tuberculosis
Structure of the NDH-2 - HQNO inhibited complex provides molecular insight into quinone-binding site inhibitors.
Tuberculosis
The structure of the yeast NADH dehydrogenase (Ndi1) reveals overlapping binding sites for water- and lipid-soluble substrates.
Tuberculosis
The type I NADH dehydrogenase of Mycobacterium tuberculosis counters phagosomal NOX2 activity to inhibit TNF-alpha-mediated host cell apoptosis.
Tuberculosis
Use of Gen-Probe AccuProbes to identify Mycobacterium avium complex, Mycobacterium tuberculosis complex, Mycobacterium kansasii, and Mycobacterium gordonae directly from BACTEC TB broth cultures.
Tuberous Sclerosis
5-Aza-2'-deoxycytidine increases hypoxia tolerance-dependent autophagy in mouse neuronal cells by initiating the TSC1/mTOR pathway.
Tuberous Sclerosis
A new homozygous HERC1 gain-of-function variant in MDFPMR syndrome leads to mTORC1 hyperactivation and reduced autophagy during cell catabolism.
Tuberous Sclerosis
Ablation of TSC2 enhances insulin secretion by increasing the number of mitochondria through activation of mTORC1.
Tuberous Sclerosis
Allosteric and ATP-Competitive Inhibitors of mTOR Effectively Suppress Tumor Progression-Associated Epithelial-Mesenchymal Transition in the Kidneys of Tsc2+/- Mice.
Tuberous Sclerosis
Alterations in expression levels of genes in p53-related pathways determined using RNA-Seq analysis in patients with breast cancer following CIK therapy.
Tuberous Sclerosis
Analysis of Proteins That Rapidly Change Upon Mechanistic/Mammalian Target of Rapamycin Complex 1 (mTORC1) Repression Identifies Parkinson Protein 7 (PARK7) as a Novel Protein Aberrantly Expressed in Tuberous Sclerosis Complex (TSC).
Tuberous Sclerosis
Antidiabetic Drug Metformin Prevents Progression of Pancreatic Cancer by Targeting in Part Cancer Stem Cells and mTOR Signaling.
Tuberous Sclerosis
Association of focal adhesion kinase with tuberous sclerosis complex 2 in the regulation of s6 kinase activation and cell growth.
Tuberous Sclerosis
Autophagy-dependent metabolic reprogramming sensitizes TSC2-deficient cells to the antimetabolite 6-aminonicotinamide.
Tuberous Sclerosis
Balanced mTORC1 activity in oligodendrocytes is required for accurate CNS myelination.
Tuberous Sclerosis
Benign clear cell "sugar" tumor of the lung in a patient with Birt-Hogg-Dubé syndrome: a case report.
Tuberous Sclerosis
Bile acid exposure up-regulates tuberous sclerosis complex 1/mammalian target of rapamycin pathway in Barrett's-associated esophageal adenocarcinoma.
Tuberous Sclerosis
Cell cycle-regulated phosphorylation of hamartin, the product of the tuberous sclerosis complex 1 gene, by cyclin-dependent kinase 1/cyclin B.
Tuberous Sclerosis
Cell-specific alterations of glutamate receptor expression in tuberous sclerosis complex cortical tubers.
Tuberous Sclerosis
Clinical and Histologic Analysis of the Efficacy of Topical Rapamycin Therapy Against Hypomelanotic Macules in Tuberous Sclerosis Complex.
Tuberous Sclerosis
Coding and small non-coding transcriptional landscape of tuberous sclerosis complex cortical tubers: implications for pathophysiology and treatment.
Tuberous Sclerosis
Comparison of the functional and structural characteristics of rare TSC2 variants with clinical and genetic findings.
Tuberous Sclerosis
Conditional knockout of Tsc1 in ROR?t-expressing cells induces brain damage and early death in mice.
Tuberous Sclerosis
Conjunctival lymphangioma in a 4-year-old girl revealed tuberous sclerosis complex.
Tuberous Sclerosis
Critical role of the tumor suppressor tuberous sclerosis complex 1 in dendritic cell activation of CD4 T cells by promoting MHC class II expression via IRF4 and CIITA.
Tuberous Sclerosis
Deficiency in pulmonary surfactant proteins in mice with fatty acid binding protein 4-Cre-mediated knockout of the tuberous sclerosis complex 1 gene.
Tuberous Sclerosis
Defining the Role of Cerebellar Purkinje Cells in Autism Spectrum Disorders.
Tuberous Sclerosis
Dissociate expression of tuberous sclerosis complex 1 product hamartin in a skin and pulmonary lesion of a tuberous sclerosis complex.
Tuberous Sclerosis
Downregulation of MicroRNA-193b-3p Promotes Autophagy and Cell Survival by Targeting TSC1/mTOR Signaling in NSC-34 Cells.
Tuberous Sclerosis
Dysregulation of Mammalian Target of Rapamycin Signaling in Mouse Models of Autism.
Tuberous Sclerosis
Dysregulation of the IGF-I/PI3K/AKT/mTOR signaling pathway in autism spectrum disorders.
Tuberous Sclerosis
Effects of combining rapamycin and resveratrol on apoptosis and growth of TSC2-deficient xenograft tumors.
Tuberous Sclerosis
Efficacy and Safety of Topical Sirolimus Therapy for Facial Angiofibromas in the Tuberous Sclerosis Complex : A Randomized Clinical Trial.
Tuberous Sclerosis
Electroacupuncture Reduces Weight in Diet-Induced Obese Rats via Hypothalamic Tsc1 Promoter Demethylation and Inhibition of the Activity of mTORC1 Signaling Pathway.
Tuberous Sclerosis
Endogenous neuroprotection: hamartin modulates an austere approach to staying alive in a recession.
Tuberous Sclerosis
Energy Stress-Mediated Cytotoxicity in Tuberous Sclerosis Complex 2-Deficient Cells with Nelfinavir and Mefloquine Treatment.
Tuberous Sclerosis
Essential role of glucokinase in the protection of pancreatic ? cells to the glucose energetic status.
Tuberous Sclerosis
G3BPs tether the TSC complex to lysosomes and suppress mTORC1 signaling.
Tuberous Sclerosis
GENETIC ACTIVATION OF mTORC1 SIGNALING WORSENS NEUROCOGNITIVE OUTCOME AFTER TRAUMATIC BRAIN INJURY.
Tuberous Sclerosis
Genetically engineered human cortical spheroid models of tuberous sclerosis.
Tuberous Sclerosis
Genome-wide CRISPR screening reveals nucleotide synthesis negatively regulates autophagy.
Tuberous Sclerosis
Ghrelin Inhibits the Differentiation of T Helper 17 Cells through mTOR/STAT3 Signaling Pathway.
Tuberous Sclerosis
Hamartin, the tuberous sclerosis complex 1 gene product, interacts with polo-like kinase 1 in a phosphorylation-dependent manner.
Tuberous Sclerosis
Hamartin: An Endogenous Neuroprotective Molecule Induced by Hypoxic Preconditioning.
Tuberous Sclerosis
Hepatic mTORC1 controls locomotor activity, body temperature, and lipid metabolism through FGF21.
Tuberous Sclerosis
Hepatitis B Virus X Protein Upregulates mTOR Signaling through IKK? to Increase Cell Proliferation and VEGF Production in Hepatocellular Carcinoma.
Tuberous Sclerosis
Hyperactivated mTORC1 downregulation of FOXO3a/PDGFR?/AKT cascade restrains tuberous sclerosis complex-associated tumor development.
Tuberous Sclerosis
Hypoxia-inducible factor-1a contributes to dendritic overgrowth in tuberous sclerosis.
Tuberous Sclerosis
Identification of an AMPK phosphorylation site in Drosophila TSC2 (gigas) that regulate cell growth.
Tuberous Sclerosis
Identification of potential drug targets for tuberous sclerosis complex by synthetic screens combining CRISPR-based knockouts with RNAi.
Tuberous Sclerosis
IGF-1 stimulates protein synthesis by enhanced signaling through mTORC1 in bovine mammary epithelial cells.
Tuberous Sclerosis
Inactivation of Tsc2 in Mesoderm-Derived Cells Causes Polycystic Kidney Lesions and Impairs Lung Alveolarization.
Tuberous Sclerosis
Inhibition of ERK1/2 Restores GSK3? Activity and Protein Synthesis Levels in a Model of Tuberous Sclerosis.
Tuberous Sclerosis
Inhibition of the mammalian target of rapamycin blocks epilepsy progression in NS-Pten conditional knockout mice.
Tuberous Sclerosis
Insulin Signaling in Bupivacaine-induced Cardiac Toxicity: Sensitization during Recovery and Potentiation by Lipid Emulsion.
Tuberous Sclerosis
Interleukin-6 mediates PSAT1 expression and serine metabolism in TSC2-deficient cells.
Tuberous Sclerosis
Isolated cortical tuber in an infant with genetically confirmed tuberous sclerosis complex 1 presenting with symptomatic West syndrome.
Tuberous Sclerosis
Leucine-Rich Glioma Inactivated 1 Promotes Oligodendrocyte Differentiation and Myelination via TSC-mTOR Signaling.
Tuberous Sclerosis
Loss of TSC complex enhances gluconeogenesis via upregulation of Dlk1-Dio3 locus miRNAs.
Tuberous Sclerosis
Loss of Tsc1 accelerates malignant gliomagenesis when combined with oncogenic signals.
Tuberous Sclerosis
Loss of Tsc1, but not Pten, in renal tubular cells causes polycystic kidney disease by activating mTORC1.
Tuberous Sclerosis
Loss of tuberous sclerosis complex 1 (Tsc1) expression results in increased Rheb/S6K pathway signaling important for astrocyte cell size regulation.
Tuberous Sclerosis
Loss of tuberous sclerosis complex 2 sensitizes tumors to nelfinavir-bortezomib therapy to intensify endoplasmic reticulum stress-induced cell death.
Tuberous Sclerosis
Lymphangioleiomyomatosis Biomarkers Linked to Lung Metastatic Potential and Cell Stemness.
Tuberous Sclerosis
Mammalian target of rapamycin complex 1 (mTORC1) enhances bortezomib-induced death in tuberous sclerosis complex (TSC)-null cells by a c-MYC-dependent induction of the unfolded protein response.
Tuberous Sclerosis
Mesangial Cell Mammalian Target of Rapamycin Complex 1 Activation Results in Mesangial Expansion.
Tuberous Sclerosis
Metabolomic studies identify changes in transmethylation and polyamine metabolism in a brain-specific mouse model of tuberous sclerosis complex.
Tuberous Sclerosis
Metformin Modulates T Cell Function and Alleviates Liver Injury Through Bioenergetic Regulation in Viral Hepatitis.
Tuberous Sclerosis
Methionine controls insulin/mammalian target of rapamycin complex 1 activity by modulating tuberous sclerosis complex 2 stability.
Tuberous Sclerosis
MicroRNA-141-3p regulates cellular proliferation, migration, and invasion in esophageal cancer by targeting tuberous sclerosis complex 1.
Tuberous Sclerosis
microRNA-19a protects osteoblasts from dexamethasone via targeting TSC1.
Tuberous Sclerosis
MicroRNA-34a activation in tuberous sclerosis complex during early brain development may lead to impaired corticogenesis.
Tuberous Sclerosis
Minireview: Lymphangioleiomyomatosis (LAM): The "Other" Steroid-Sensitive Cancer.
Tuberous Sclerosis
miR-125b-5p/STAT3 Pathway Regulated by mTORC1 Plays a Critical Role in Promoting Cell Proliferation and Tumor Growth.
Tuberous Sclerosis
MiR-126-3p promotes the cell proliferation and inhibits the cell apoptosis by targeting TSC1 in the porcine granulosa cells.
Tuberous Sclerosis
MiR-451 is decreased in hypertrophic cardiomyopathy and regulates autophagy by targeting TSC1.
Tuberous Sclerosis
Moderate lifelong overexpression of tuberous sclerosis complex 1 (TSC1) improves health and survival in mice.
Tuberous Sclerosis
mTOR complex 1 signalling regulates the balance between lipid synthesis and oxidation in hypoxia lymphocytes.
Tuberous Sclerosis
mTORC1 activation downregulates FGFR3 and PTH/PTHrP receptor in articular chondrocytes to initiate osteoarthritis.
Tuberous Sclerosis
mTORC1 activation in lung mesenchyme drives sex- and age-dependent pulmonary structure and function decline.
Tuberous Sclerosis
mTORC1 Activation in Osteoclasts Prevents Bone Loss in a Mouse Model of Osteoporosis.
Tuberous Sclerosis
MTORC1 coordinates the autophagy and apoptosis signaling in articular chondrocytes in osteoarthritic temporomandibular joint.
Tuberous Sclerosis
Mutation of the co-chaperone Tsc1 in bladder cancer diminishes Hsp90 acetylation and reduces drug sensitivity and selectivity.
Tuberous Sclerosis
N-Formyl-3,4-methylenedioxy-benzylidene-gamma-butyrolaetam, KNK437 induces caspase-3 activation through inhibition of mTORC1 activity in Cos-1 cells.
Tuberous Sclerosis
Noncanonical Inhibition of mTORC1 by Coxiella burnetii Promotes Replication within a Phagolysosome-Like Vacuole.
Tuberous Sclerosis
One-Hit Wonders and 2-Hit Tubers: A Second-Hit to TSC2 Causes Tuber-Like Cells in Spheroids.
Tuberous Sclerosis
Osteocyte TSC1 promotes sclerostin secretion to restrain osteogenesis in mice.
Tuberous Sclerosis
p53 haploinsufficiency and increased mTOR signaling define a subset of aggressive hepatocellular carcinoma.
Tuberous Sclerosis
Pancreatic Neuroendocrine Tumor in a Young Child With Tuberous Sclerosis Complex 1.
Tuberous Sclerosis
Pathological Findings of a Subependymal Giant Cell Astrocytoma Following Treatment With Rapamycin.
Tuberous Sclerosis
Periungual fibroma (Koenen tumors) as isolated sign of tuberous sclerosis complex with tuberous sclerosis complex 1 germline mutation.
Tuberous Sclerosis
Pharmacological inhibition of Polo-like kinase 1 (PLK1) by BI-2536 decreases the viability and survival of hamartin and tuberin deficient cells via induction of apoptosis and attenuation of autophagy.
Tuberous Sclerosis
Phenotypic Screen with TSC-Deficient Neurons Reveals Heat-Shock Machinery as a Druggable Pathway for mTORC1 and Reduced Cilia.
Tuberous Sclerosis
Pivotal role of augmented ?B-crystallin in tumor development induced by deficient TSC1/2 complex.
Tuberous Sclerosis
Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa).
Tuberous Sclerosis
Podocytes maintain high basal levels of autophagy independent of mtor signaling.
Tuberous Sclerosis
Postnatal reduction of tuberous sclerosis complex 1 expression in astrocytes and neurons causes seizures in an age-dependent manner.
Tuberous Sclerosis
PRAS40 and PRR5-Like Protein Are New mTOR Interactors that Regulate Apoptosis.
Tuberous Sclerosis
Prevention of alveolar destruction and airspace enlargement in a mouse model of pulmonary lymphangioleiomyomatosis (LAM).
Tuberous Sclerosis
Prolonging the survival of Tsc2 conditional knockout mice by glutamine supplementation.
Tuberous Sclerosis
Promoting axon regeneration in the adult CNS by modulation of the PTEN/mTOR pathway.
Tuberous Sclerosis
Pulmonary lymphangioleiomyomatosis (LAM): progress and current challenges.
Tuberous Sclerosis
Rapamycin reverses impaired social interaction in mouse models of tuberous sclerosis complex.
Tuberous Sclerosis
Regulation of mast cell survival and function by tuberous sclerosis complex 1.
Tuberous Sclerosis
Renal tumours in a Tsc2(+/-) mouse model do not show feedback inhibition of Akt and are effectively prevented by rapamycin.
Tuberous Sclerosis
Reprogramming towards anabolism impedes degeneration in a preclinical model of retinitis pigmentosa.
Tuberous Sclerosis
Response to everolimus is seen in TSC-associated SEGAs and angiomyolipomas independent of mutation type and site in TSC1 and TSC2.
Tuberous Sclerosis
Resveratrol prevents rapamycin-induced upregulation of autophagy and selectively induces apoptosis in TSC2-deficient cells.
Tuberous Sclerosis
Review of the treatment options for epilepsy in tuberous sclerosis complex: towards precision medicine.
Tuberous Sclerosis
Rheb activation in subventricular zone progenitors leads to heterotopia, ectopic neuronal differentiation, and rapamycin-sensitive olfactory micronodules and dendrite hypertrophy of newborn neurons.
Tuberous Sclerosis
Rheb1 deletion in myeloid cells aggravates OVA-induced allergic inflammation in mice.
Tuberous Sclerosis
Role of I?B kinase-? in the growth-promoting effects of angiotensin II in vitro and in vivo.
Tuberous Sclerosis
Role of Mechanistic Target of Rapamycin and Autophagy in Alcohol-Induced Adipose Atrophy and Liver Injury.
Tuberous Sclerosis
Role of Neurofilament Light Polypeptide in Head and Neck Cancer Chemoresistance.
Tuberous Sclerosis
Role of the TSC1-TSC2 Complex in the Integration of Insulin and Glucose Signaling Involved in Pancreatic {beta}-Cell Proliferation.
Tuberous Sclerosis
Role of tumor suppressor TSC1 in regulating antigen-specific primary and memory CD8 T cell responses to bacterial infection.
Tuberous Sclerosis
Selective Tuberous Sclerosis Complex 1 Gene Deletion in Smooth Muscle Activates Mammalian Target of Rapamycin Signaling and Induces Pulmonary Hypertension.
Tuberous Sclerosis
Sirolimus for epilepsy in children with tuberous sclerosis complex: A randomized controlled trial.
Tuberous Sclerosis
Smooth muscle protein-22-mediated deletion of Tsc1 results in cardiac hypertrophy that is mTORC1-mediated and reversed by rapamycin.
Tuberous Sclerosis
Specific disruption of Tsc1 in ovarian granulosa cells promotes ovulation and causes progressive accumulation of corpora lutea.
Tuberous Sclerosis
Stability of tuberous sclerosis complex 2 is controlled by methylation at R1457 and R1459.
Tuberous Sclerosis
Structural Basis of the Interaction between Tuberous Sclerosis Complex 1 (TSC1) and Tre2-Bub2-Cdc16 Domain Family Member 7 (TBC1D7).
Tuberous Sclerosis
Sturge Weber-Like Gyral Calcification Seen in Tuberous Sclerosis Complex 1.
Tuberous Sclerosis
Synovial macrophage M1 polarisation exacerbates experimental osteoarthritis partially through R-spondin-2.
Tuberous Sclerosis
Target-of-rapamycin complex 1 (Torc1) signaling modulates cilia size and function through protein synthesis regulation.
Tuberous Sclerosis
Targeted Approaches toward Understanding and Treating Pulmonary Lymphangioleiomyomatosis (LAM).
Tuberous Sclerosis
Targeting protein homeostasis with nelfinavir/salinomycin dual therapy effectively induces death of mTORC1 hyperactive cells.
Tuberous Sclerosis
Targeting the phosphatidylinositol 3-kinase pathway in airway smooth muscle: rationale and promise.
Tuberous Sclerosis
TFEB activation restores migration ability to Tsc1-deficient adult neural stem/progenitor cells.
Tuberous Sclerosis
The Characterization of a Subependymal Giant Astrocytoma-Like Cell Line from Murine Astrocyte with mTORC1 Hyperactivation.
Tuberous Sclerosis
The Circadian Protein Period2 Suppresses mTORC1 Activity via Recruiting Tsc1 to mTORC1 Complex.
Tuberous Sclerosis
The coding and non-coding transcriptional landscape of subependymal giant cell astrocytomas.
Tuberous Sclerosis
The FoxO-BNIP3 axis exerts a unique regulation of mTORC1 and cell survival under energy stress.
Tuberous Sclerosis
The GID ubiquitin ligase complex is a regulator of AMPK activity and organismal lifespan.
Tuberous Sclerosis
The mammalian target of rapamycin-signaling pathway in regulating metabolism and growth.
Tuberous Sclerosis
The mTORC1 effectors S6K1 and 4E-BP play different roles in CNS axon regeneration.
Tuberous Sclerosis
The p53 target Plk2 interacts with TSC proteins impacting mTOR signaling, tumor growth and chemosensitivity under hypoxic conditions.
Tuberous Sclerosis
The physiological mTOR complex 1 inhibitor DDIT4 mediates therapy resistance in glioblastoma.
Tuberous Sclerosis
The Role of TSC1 in the Macrophages Against Vibrio vulnificus Infection.
Tuberous Sclerosis
The Stress-Induced Atf3-Gelsolin Cascade Underlies Dendritic Spine Deficits in Neuronal Models of Tuberous Sclerosis Complex.
Tuberous Sclerosis
The TSC1-mTOR-PLK axis regulates the homeostatic switch from Schwann cell proliferation to myelination in a stage-specific manner.
Tuberous Sclerosis
The Tsc1-Tsc2 complex influences neuronal polarity by modulating TORC1 activity and SAD levels.
Tuberous Sclerosis
The tuberous sclerosis complex subunit TBC1D7 is stabilized by Akt phosphorylation-mediated 14-3-3 binding.
Tuberous Sclerosis
Transforming Growth Factor-? Signaling Participates in the Maintenance of the Primordial Follicle Pool in the Mouse Ovary.
Tuberous Sclerosis
Treatment of Renal Angiomyolipoma and Other Hamartomas in Patients with Tuberous Sclerosis Complex.
Tuberous Sclerosis
TSC but not PTEN loss in starving cones of retinitis pigmentosa mice leads to an autophagy defect and mTORC1 dissociation from the lysosome.
Tuberous Sclerosis
Tsc1 (hamartin) confers neuroprotection against ischemia by inducing autophagy.
Tuberous Sclerosis
TSC1 and TSC2 regulate cilia length and canonical Hedgehog signaling via different mechanisms.
Tuberous Sclerosis
TSC1 controls IL-1? expression in macrophages via mTORC1-dependent C/EBP? pathway.
Tuberous Sclerosis
TSC1 deletion in fibroblasts alleviates lipopolysaccharide-induced acute kidney injury.
Tuberous Sclerosis
Tsc1 expression by dendritic cells is required to preserve T-cell homeostasis and response.
Tuberous Sclerosis
TSC1 Promotes B Cell Maturation but Is Dispensable for Germinal Center Formation.
Tuberous Sclerosis
TSC1 regulates osteoclast podosome organization and bone resorption through mTORC1 and Rac1/Cdc42.
Tuberous Sclerosis
Tsc1-dependent transcriptional programming of dendritic cell homeostasis and function.
Tuberous Sclerosis
Tsc1-mTORC1 signaling controls striatal dopamine release and cognitive flexibility.
Tuberous Sclerosis
TSC2 regulates lysosome biogenesis via a non-canonical RAGC and TFEB-dependent mechanism.
Tuberous Sclerosis
Tuberous sclerosis 1 (Tsc1) mediated mTORC1 activation promotes glycolysis in tubular epithelial cells in kidney fibrosis.
Tuberous Sclerosis
Tuberous sclerosis complex 1 regulates dE2F1 expression during development and cooperates with RBF1 to control proliferation and survival.
Tuberous Sclerosis
Tuberous sclerosis complex 1-mechanistic target of rapamycin complex 1 signaling determines brown-to-white adipocyte phenotypic switch.
Tuberous Sclerosis
Tuberous Sclerosis Complex 1: An Epithelial Tumor Suppressor Essential to Prevent Spontaneous Prostate Cancer in Aged Mice.
Tuberous Sclerosis
Tuberous sclerosis complex inactivation disrupts melanogenesis via mTORC1 activation.
Tuberous Sclerosis
Tuberous sclerosis complex, mTOR, and the kidney: report of an NIDDK-sponsored workshop.
Tuberous Sclerosis
Unrestrained mammalian target of rapamycin complexes 1 and 2 increase expression of phosphatase and tensin homolog deleted on chromosome 10 to regulate phosphorylation of Akt kinase.
Tuberous Sclerosis
Upregulation of 6-phosphofructo-2-kinase (PFKFB3) by hyperactivated mammalian target of rapamycin complex 1 is critical for tumor growth in tuberous sclerosis complex.
Tuberous Sclerosis
Vitamin D increases glucocorticoid efficacy via inhibition of mTORC1 in experimental models of multiple sclerosis.
Tuberous Sclerosis
Vps34-mediated macropinocytosis in Tuberous Sclerosis Complex 2-deficient cells supports tumorigenesis.
Tuberous Sclerosis
ZRF1 is a novel S6 kinase substrate that drives the senescence programme.
Tuberous Sclerosis
[Mechanism of Electropuncture for Reducing Diet-induced Obesity Rat Weight through Hypothala- mus TSC1 -mTOR Signal Pathway].
Tuberous Sclerosis
[Tsc1 promoter methylation rate, mTOR expression in food-induced obese rat hypothalamus].
Typhoid Fever
Protection of chickens against experimental fowl typhoid using a nuoG mutant of Salmonella serotype Gallinarum.
Ureteral Obstruction
Rotenone remarkably attenuates oxidative stress, inflammation, and fibrosis in chronic obstructive uropathy.
Urinary Bladder Neoplasms
Mesenchymal stem cells-derived exosomal microRNA-139-5p restrains tumorigenesis in bladder cancer by targeting PRC1.
Urinary Bladder Neoplasms
Rapamycin Prevents Surgery-Induced Immune Dysfunction in Patients with Bladder Cancer.
Urinary Bladder Neoplasms
Synthesis and antitumor activities of transition metal complexes of a bis-Schiff base of 2-hydroxy-1-naphthalenecarboxaldehyde.
Urinary Tract Infections
Function of GRIM-19, a Mitochondrial Respiratory Chain Complex I Protein, in Innate Immunity.
Uterine Cervical Neoplasms
Downregulation of microRNA-425-5p suppresses cervical cancer tumorigenesis by targeting AIFM1.
Uterine Cervical Neoplasms
GRIM-19 Disrupts E6/E6AP Complex to Rescue p53 and Induce Apoptosis in Cervical Cancers.
Uterine Cervical Neoplasms
GRIM-19 Restores Cervical Cancer Cell Senescence by Repressing hTERT Transcription.
Uterine Cervical Neoplasms
Identification and characterization of genes involved in the carcinogenesis of human squamous cell cervical carcinoma.
Uterine Cervical Neoplasms
Investigation of in vitro anticancer and DNA strap interactions in live cells using carboplatin type Cu(II) and Zn(II) metalloinsertors.
Uterine Cervical Neoplasms
Monofunctional platinum complexes containing a 4-nitrobenzo-2-oxa-1,3-diazole fluorophore: Distribution in tumour cells.
Uterine Cervical Neoplasms
Oxoplatin-B, a cisplatin-based platinum(IV) complex with photoactive BODIPY for mitochondria specific "chemo-PDT" activity.
Uterine Cervical Neoplasms
Photocytotoxic 3d-metal scorpionates with a 1,8-naphthalimide chromophore showing photoinduced DNA and protein cleavage activity.
Uterine Cervical Neoplasms
Synthesis, crystal structure and biological activity of two Mn complexes with 4-acyl pyrazolone derivatives.
Uterine Cervical Neoplasms
Synthesis, structures, nuclease activity, cytotoxicity, DFT and molecular docking studies of two nitrato bridged homodinuclear (Cu-Cu, Zn-Zn) complexes containing 2,2'-bipyridine and a chalcone derivative.
Vascular Calcification
Defective Interplay Between mTORC1 Activity and Endoplasmic Reticulum Stress-Unfolded Protein Response in Uremic Vascular Calcification.
Vascular Malformations
EPHB4 Mutation Implicated in Capillary Malformation-Arteriovenous Malformation Syndrome: A Case Report.
Vascular System Injuries
Wild-type p53-induced phosphatase 1 promotes vascular smooth muscle cell proliferation and neointima hyperplasia after vascular injury via p-adenosine 5'-monophosphate-activated protein kinase/mammalian target of rapamycin complex 1 pathway.
Vasculitis
Circulating nicotinamide adenine dinucleotide-ubiquinone oxidoreductase chain 6 is associated with disease activity of anti-neutrophil cytoplasmic antibody-associated vasculitis.
Ventricular Dysfunction
Complex I syndrome in myocardial stunning and the effect of adenosine.
Ventricular Dysfunction, Left
TNF-induced mitochondrial damage: a link between mitochondrial complex I activity and left ventricular dysfunction.
Vesicular Stomatitis
Glucosamine promotes hepatitis B virus replication through its dual effects in suppressing autophagic degradation and inhibiting MTORC1 signaling.
Vesicular Stomatitis
Traffic of Kv4 K+ channels mediated by KChIP1 is via a novel post-ER vesicular pathway.
Virus Diseases
A tightly regulated IL-22 response maintains immune functions and homeostasis in systemic viral infection.
Virus Diseases
GRIM-19 Restricts HCV Replication by Attenuating Intracellular Lipid Accumulation.
Virus Diseases
Mammalian target of rapamycin complex 1 signalling is essential for germinal centre reaction.
Virus Diseases
mTORC1 Negatively Regulates the Replication of Classical Swine Fever Virus Through Autophagy and IRES-Dependent Translation.
Vision Disorders
Visual function, ocular motility and ocular characteristics in patients with mitochondrial complex I deficiency.
Vitamin A Deficiency
Effects of vitamin A deficiency on mitochondrial function in rat liver and heart.
Vitamin D Deficiency
Diet-induced vitamin D deficiency reduces skeletal muscle mitochondrial respiration.
Waldenstrom Macroglobulinemia
Dual targeting of the PI3K/Akt/mTOR pathway as an antitumor strategy in Waldenstrom macroglobulinemia.
Werner Syndrome
Familial non-medullary thyroid carcinoma: clinico-pathological features, current knowledge and novelty regarding genetic risk factors.
Whooping Cough
A(2B) adenosine receptors inhibit superoxide production from mitochondrial complex I in rabbit cardiomyocytes via a mechanism sensitive to Pertussis toxin.
Wilms Tumor
Mutations in microRNA processing genes in Wilms tumors derepress the IGF2 regulator PLAG1.
Zellweger Syndrome
The use of targeted genomic capture and massively parallel sequencing in diagnosis of Chinese Leukoencephalopathies.
Zika Virus Infection
Mechanistic Target of Rapamycin (mTOR) Signaling Activation Antagonizes Autophagy to Facilitate Zika Virus Replication.
[pyruvate dehydrogenase (acetyl-transferring)]-phosphatase deficiency
A Diagnostic Algorithm for Mitochondrial Disorders in Estonian Children.
[pyruvate dehydrogenase (acetyl-transferring)]-phosphatase deficiency
The expanding clinical spectrum of mitochondrial diseases.
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Ki VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
0.00104
2-n-heptyl-4-hydroxyquinoline-N-oxide
pH 7.0, 30°C, N-terminally tagged protein
0.00075
2-n-heptyl-4-hydroxyquinoline-N-oxide
pH 7.0, 30°C, N-terminally tagged protein
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IC50 VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
0.017
1-(4-(4-nitrophenyl)piperazin-1-yl)-4-(thiophen-2-yl)butan-1-one
Homo sapiens
-
pH not specified in the publication, temperature not specified in the publication
-
0.014
3-(thiophen-2-ylthio)-1-(4-(4-(trifluoromethyl)phenyl)piperazin-1-yl)propan-1-one
Homo sapiens
-
pH not specified in the publication, temperature not specified in the publication
-
0.027
4-(thiophen-2-yl)-1-(4-(4-(trifluoromethyl)phenyl)piperazin-1-yl)butan-1-one
Homo sapiens
-
pH not specified in the publication, temperature not specified in the publication
-
0.015
4-(thiophen-2-yl)-1-(4-(4-(trifluoromethyl)phenyl)piperidin-1-yl)butan-1-one
Homo sapiens
-
pH not specified in the publication, temperature not specified in the publication
-
0.006
5-phenyl-1-(4-(4-(trifluoromethyl)phenyl)piperazin-1-yl)pentan-1-one
Homo sapiens
-
pH not specified in the publication, temperature not specified in the publication
-
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
-
in the high positive schizophrenic group a positive correlation exists between cerebral glucose metabolism and complex I activity in the basal ganglia (lentiform nucleus including the putamen and globus pallidus) and the thalamus, but reaches significance only for its left side
brenda
-
in the low positive schizophrenic group, a region of negative correlation between cerebral glucose metabolism and complex I activity is identified bilaterally in the cerebellum and brainstem
brenda
-
in the low positive schizophrenic group, a region of negative correlation between cerebral glucose metabolism and complex I activity is identified bilaterally in the cerebellum and brainstem
brenda
-
in the high positive schizophrenic group a positive correlation exists between cerebral glucose metabolism and complex I activity in the basal ganglia (lentiform nucleus including the putamen and globus pallidus) and the thalamus, but reaches significance only for its left side
brenda
additional information
-
complex I activity is significantly higher in high positive schizophrenics as compared to control subjects and low positive schizophrenic patients. In low positive schizophrenic patients complex I activity does not differ from that of the control group. Entering age and gender as covariates has no effect on the significance of difference in complex I activity between subject groups. Significant positive correlation between complex I activity and the severity of positive symptoms
brenda
-
NADH dehydrogenase activity in the platelets of patients with Parkinson's disease from an Indian population is lower than that in healthy age and gender-matched controls
brenda
-
severe decrease of complex I activity in a patient with Leigh syndrome and an elevated complex IV/complex I activity ratio
brenda
-
complex I activity in cultured skin fibroblasts is in the normal range in a patient with Leigh syndrome, but complex IV/complex I activity ratio is significantly elevated and complex I/citrate synthase is decreased
brenda
-
fully assembled complex I is decreased in patient skin fibroblasts
brenda
additional information
-
in the control group, no areas of significant positive or negative correlation between cerebral glucose metabolism and peripheral complex I activity exist
brenda
additional information
-
transmitochondrial cell line, cybrids from a patient with a multisystem mitochondrial disorder with a G6930A nonsense mutation in the COX I gene. G6930A mutation causes a disruption in the assembly and defective activity of complex VI
brenda
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
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GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
physiological function
after reconstituting isolated AIFM2 into bacterial or mitochondrial membranes, N-terminally tagged AIFM2 displays substantial NADH:O2 activity and supports NADH-linked proton pumping activities in the host membranes. Overexpressing N-terminally tagged AIFM1 and AIFM2 enhances the growth of a double knock-out Escherichia coli strain lacking complex I and NDH-2. NADH-binding site mutants of N-terminally tagged AIFM1 and AIFM2 fail to show both NADH:O2 activity and the growth-enhancing effect
malfunction
-
defects in complex I are the most frequent cause of human respiratory disorders. Substitution L158P does not lead to any respiratory enzyme defects when present in the heteroplasmic state in a patient with chronic progressive external ophthalmoplegia
physiological function
after reconstituting isolated AIFM1 into bacterial or mitochondrial membranes, N-terminally tagged AIFM1 displays substantial NADH:O2 activity and supports NADH-linked proton pumping activities in the host membranes. Overexpressing N-terminally tagged AIFM1 and AIF-homologous mitochondrion-associated inducer of death AMID enhances the growth of a double knock-out Escherichia coli strain lacking complex I and NDH-2. C-terminally tagged AIFM1 and NADH-binding site mutants of N-terminally tagged AIFM1 and AMID fail to show both NADH:O2 activity and the growth-enhancing effect
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UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable).
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable). Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
SUBUNIT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
additional information
additional information
-
the enzyme complex consists of at least 43 proteins, seven are encoded by the mitochondrial genome, while the remainder are encoded by the nuclear genome
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PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
additional information
-
mutation in the first intron of the NDUFS7 gene (c.17-1167 C > G), creates a strong donor splice site resulting in the generation of a cryptic exon, shows marked decrease of fully assembled complex I
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PURIFICATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
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CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
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APPLICATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
drug development
-
nucleoside analog reverse transcriptase inhibitors are capable of affecting complex I activity in a non-polymerase-gamma/mtDNA mediated pathway. Elevations in superoxide produced at complex I caused by nucleoside analog reverse transcriptase inhibitors can provide a mechanism for the oxidative stress observed with these drugs
medicine
additional information
medicine
-
a deficient activity of the NADH:ubiquinone oxidoreductase enzyme complex is frequently observed in clinical heterogeneous group of mitochondrial disorders with Leigh (-like) disease as the main contributor. Enzyme complex activity measurement in skeletal muscle is the mainstay of the diagnostic process. Fibroblast studies are a prerequisite whenever prenatal enzyme diagnostic is considered
medicine
-
a systemic defect in complex I activity is not present in early Huntington's disease when striatal neuronal degeneration is already present
medicine
-
complex I deficient patients show a specific increase in superoxide dismutase activity. Reactive oxygen species production remains significantly elevated in complex I deficient patients compared to controls, whereas glutathione peroxidase, catalase and glutathione-S-transferase are significantly reduced. Information on the status of reactive oxygen species and marking the alteration of reactive oxygen species scavenging enzymes in peripheral lymphocytes or lymphoblast cell lines will provide a better way to design antioxidant therapies for such disorders like complex I deficiency
medicine
-
correlation between peripheral complex I activity and cerebral glucose metabolism in regions implicated in schizophrenia, may be a pathological factor that is differentially expressed in subgroups of schizophrenic patients
medicine
-
identification of a NDUFS7 mutation in a consanguineous family with Leigh syndrome and isolated complex I deficiency
medicine
-
superoxide production is increased in children with inherited complex I deficiency, which is primarily a consequence of the reduction in cellular complex I activity and not of a further leakage of electrons from mutationally malformed complexes
medicine
-
cellular levels of reactive oxygen species are significantly increased in cells of patients with DNA-inherited isolated complex I deficiency. This increase is associated with variable decreases in the amount and intrinsic catalytic activity of complex I. Chronic treatment with a vitamin E derivative (Trolox) increases the amount of complex I and might provide an experimental basis for the use of antioxidants to treat complex I deficient patients
medicine
-
cellular levels of reactive oxygen species are significantly increased in cells of patients with nDNA-inherited isolated complex I deficiency. This increase is associated with variable decreases in the amount and intrinsic catalytic activity of complex I. Chronic treatment with a vitamin E derivative (Trolox) increases the amount of complex I and might provide an experimental basis for the use of antioxidants to treat complex I deficient patients
medicine
-
mutations in NADH dehydrogenase ND subunits of complex I are an important genetic cause of childhood mitochondrial encephalopathies
medicine
-
NADH dehydrogenase activity in the platelets of patients with Parkinson's disease from an Indian population is lower than that in healthy age and gender-matched controls. The study contribute to the evidence of mitochondrial dysfunction playing a major role in the disease mechanism of Parkinson's disease and opens avenues for therapeutic intervention
medicine
-
alterations in mitochondrial membrane complex MMC-I may constitute an inheritable risk factor for endometriosis. MMC-I expression in eutopic endometria of patients is elevated compared to controls. Haplotype 10398A/10400C/13603AG and haplogroup N show higher endometriosis risk
additional information
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an assembled complex IV helps to maintain complex I in mammalian cells
additional information
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crucial role for mitochondrial nitric oxide synthase in oxidative stress caused by mitochondrial complex I inactivation
additional information
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reduction in complex I activity results in a decrease in mitochondrial movement which is compensated for by an increase in mitochondrial length and degree of branching and an enhanced diffusion of matrix constituents
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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Smeitink, J.; Sengers, R.; Trijbels, F.; Van den Heuvel, L.
Human NADH:ubiquinone oxidoreductase
J. Bioenerg. Biomembr.
33
259-266
2001
Homo sapiens
brenda
Shimada, T.; Moriuchi, R.; Mori, T.; Yamada, K.; Ishimaru, T.; Katamine, S.
Identification of NADH dehydrogenase 1 alpha subcomplex 5 capable to transform murine fibroblasts and overexpressed in human cervical carcinoma cell lines
Biochem. Biophys. Res. Commun.
339
852-857
2006
Homo sapiens
brenda
Huang, G.; Chen, Y.; Lu, H.; Cao, X.
Coupling mitochondrial respiratory chain to cell death: an essential role of mitochondrial complex I in the interferon-beta and retinoic acid-induced cancer cell death
Cell Death Differ.
14
327-337
2007
Homo sapiens
brenda
Sarkar, M.; Das, S.; Bandyopadhaya, A.; Ray, K.; Chaudhuri, K.
Upregulation of human mitochondrial NADH dehydrogenase subunit 5 in intestinal epithelial cells is modulated by Vibrio cholerae pathogenesis
FEBS Lett.
579
3449-3460
2005
Homo sapiens
brenda
Gabaldon, T.; Rainey, D.; Huynen, M.A.
Tracing the evolution of a large protein complex in the eukaryotes, NADH:ubiquinone oxidoreductase (Complex I)
J. Mol. Biol.
348
857-870
2005
Anopheles gambiae, Arabidopsis thaliana, Bos taurus, Saccharomyces cerevisiae, Caenorhabditis elegans, Candida albicans, Yarrowia lipolytica, Chlamydomonas reinhardtii, Drosophila melanogaster, Homo sapiens, Mus musculus, Neurospora crassa, Plasmodium falciparum, Schizosaccharomyces pombe, Takifugu rubripes, Cryptosporidium hominis, Encephalitozoon cuniculi, Guillardia theta
brenda
Parihar, M.S.; Parihar, A.; Villamena, F.A.; Vaccaro, P.S.; Ghafourifar, P.
Inactivation of mitochondrial respiratory chain complex I leads mitochondrial nitric oxide synthase to become pro-oxidative
Biochem. Biophys. Res. Commun.
367
761-767
2008
Homo sapiens, Rattus norvegicus
brenda
Koopman, W.J.; Hink, M.A.; Verkaart, S.; Visch, H.J.; Smeitink, J.A.; Willems, P.H.
Partial complex I inhibition decreases mitochondrial motility and increases matrix protein diffusion as revealed by fluorescence correlation spectroscopy
Biochim. Biophys. Acta
1767
940-947
2007
Homo sapiens
brenda
Verkaart, S.; Koopman, W.J.; van Emst-de Vries, S.E.; Nijtmans, L.G.; van den Heuvel, L.W.; Smeitink, J.A.; Willems, P.H.
Superoxide production is inversely related to complex I activity in inherited complex I deficiency
Biochim. Biophys. Acta
1772
373-381
2007
Homo sapiens
brenda
Wani, A.A.; Rangrez, A.Y.; Kumar, H.; Bapat, S.A.; Suresh, C.G.; Barnabas, S.; Patole, M.S.; Shouche, Y.S.
Analysis of reactive oxygen species and antioxidant defenses in complex I deficient patients revealed a specific increase in superoxide dismutase activity
Free Radic. Res.
42
415-427
2008
Homo sapiens
brenda
Li, Y.; DAurelio, M.; Deng, J.H.; Park, J.S.; Manfredi, G.; Hu, P.; Lu, J.; Bai, Y.
An assembled complex IV maintains the stability and activity of complex I in mammalian mitochondria
J. Biol. Chem.
282
17557-17562
2007
Homo sapiens, Mus musculus
brenda
Watabe, M.; Nakaki, T.
Mitochondrial complex I inhibitor rotenone-elicited dopamine redistribution from vesicles to cytosol in human dopaminergic SH-SY5Y cells
J. Pharmacol. Exp. Ther.
323
499-507
2007
Homo sapiens
brenda
Lebon, S.; Minai, L.; Chretien, D.; Corcos, J.; Serre, V.; Kadhom, N.; Steffann, J.; Pauchard, J.Y.; Munnich, A.; Bonnefont, J.P.; Roetig, A.
A novel mutation of the NDUFS7 gene leads to activation of a cryptic exon and impaired assembly of mitochondrial complex I in a patient with Leigh syndrome
Mol. Genet. Metab.
92
104-108
2007
Homo sapiens
brenda
Powers, W.J.; Haas, R.H.; Le, T.; Videen, T.O.; Hershey, T.; McGee-Minnich, L.; Perlmutter, J.S.
Normal platelet mitochondrial complex I activity in Huntingtons disease
Neurobiol. Dis.
27
99-101
2007
Homo sapiens
brenda
Ben-Shachar, D.; Bonne, O.; Chisin, R.; Klein, E.; Lester, H.; Aharon-Peretz, J.; Yona, I.; Freedman, N.
Cerebral glucose utilization and platelet mitochondrial complex I activity in schizophrenia: A FDG-PET study
Prog. Neuropsychopharmacol. Biol. Psychiatry
31
807-813
2007
Homo sapiens
brenda
Lund, K.C.; Wallace, K.B.
Adenosine 3,5-cyclic monophosphate (cAMP)-dependent phosphoregulation of mitochondrial complex I is inhibited by nucleoside reverse transcriptase inhibitors
Toxicol. Appl. Pharmacol.
226
94-106
2008
Homo sapiens
brenda
Lin, S.S.; Kerscher, S.; Saleh, A.; Brandt, U.; Gross, U.; Bohne, W.
The Toxoplasma gondii type-II NADH dehydrogenase TgNDH2-I is inhibited by 1-hydroxy-2-alkyl-4(1H)quinolones
Biochim. Biophys. Acta
1777
1455-1462
2008
Homo sapiens
brenda
Koopman, W.J.; Verkaart, S.; van Emst-de Vries, S.E.; Grefte, S.; Smeitink, J.A.; Nijtmans, L.G.; Willems, P.H.
Mitigation of NADH:ubiquinone oxidoreductase deficiency by chronic Trolox treatment
Biochim. Biophys. Acta
1777
853-859
2008
Homo sapiens
brenda
Willems, P.H.; Valsecchi, F.; Distelmaier, F.; Verkaart, S.; Visch, H.J.; Smeitink, J.A.; Koopman, W.J.
Mitochondrial Ca2+ homeostasis in human NADH:ubiquinone oxidoreductase deficiency
Cell Calcium
44
123-133
2008
Homo sapiens
brenda
Willems, P.H.; Smeitink, J.A.; Koopman, W.J.
Mitochondrial dynamics in human NADH:ubiquinone oxidoreductase deficiency
Int. J. Biochem. Cell Biol.
41
1773-1782
2009
Homo sapiens
brenda
Lim, B.C.; Park, J.D.; Hwang, H.; Kim, K.J.; Hwang, Y.S.; Chae, J.H.; Cheon, J.E.; Kim, I.O.; Lee, R.; Moon, H.K.
Mutations in ND subunits of complex I are an important genetic cause of childhood mitochondrial encephalopathies
J. Child Neurol.
24
828-832
2009
Homo sapiens
brenda
Varghese, M.; Pandey, M.; Samanta, A.; Gangopadhyay, P.K.; Mohanakumar, K.P.
Reduced NADH coenzyme Q dehydrogenase activity in platelets of Parkinsons disease, but not Parkinson plus patients, from an Indian population
J. Neurol. Sci.
279
39-42
2009
Homo sapiens
brenda
Larosa, V.; Coosemans, N.; Motte, P.; Bonnefoy, N.; Remacle, C.
Reconstruction of a human mitochondrial complex I mutation in the unicellular green alga Chlamydomonas
Plant J.
70
759-768
2012
Chlamydomonas sp., Homo sapiens
brenda
Elguindy, M.M.; Nakamaru-Ogiso, E.
Apoptosis-inducing factor (AIF) and its family member protein, AMID, are rotenone-sensitive NADH ubiquinone oxidoreductases (NDH-2)
J. Biol. Chem.
290
20815-20826
2015
Homo sapiens (O95831), Homo sapiens (Q9BRQ8), Homo sapiens
brenda
Govatati, S.; Deenadayal, M.; Shivaji, S.; Bhanoori, M.
Mitochondrial NADH ubiquinone oxidoreductase alterations are associated with endometriosis
Mitochondrion
13
782-790
2013
Homo sapiens
brenda
Devine, R.; Kelada, M.; Leonard, S.; Martin, D.S.D.; Walsh, J.M.D.; Breen, C.J.; Driver, R.B.; Kinsella, G.K.; Findlay, J.B.C.; Stephens, J.C.
Design, synthesis, and biological evaluation of aryl piperazines with potential as antidiabetic agents via the stimulation of glucose uptake and inhibition of NADH ubiquinone oxidoreductase
Eur. J. Med. Chem.
202
112416
2020
Homo sapiens
brenda
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