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Literature summary extracted from

  • Tretter, L.; Adam-Vizi, V.
    alpha-Ketoglutarate dehydrogenase: a target and generator of oxidative stress (2005), Philos. Trans. R. Soc. Lond. B Biol. Sci., 360, 2335-2345.
    View publication on PubMedView publication on EuropePMC

Application

EC Number Application Comment Organism
1.2.1.105 medicine altered in the post-mortem substantia nigra samples from patients suffering from Parkinson's disease, decreased activity in the brain of Alzheimer's disease patients, involving regions affected by the disease as well as regions that remain normal, reduced activity or increased vulnerability to oxidative stress in fibroblasts from patients with presenlilin-1 mutation Homo sapiens
1.2.1.105 medicine plays a role in neurodegenerative diseases Rattus norvegicus
1.2.1.105 additional information in post-mortem mice brain samples the activity is quickly lost, whereas the activity of another TPP-dependent enzyme, PDH, remains unalterd for at least 24 h Mus musculus

Protein Variants

EC Number Protein Variants Comment Organism
1.2.1.105 additional information heterozygous knockout mice deficient in the E3 subunit produce significantly less H2O2 than mitochondria form their littermate wild-type mice Mus musculus

Inhibitors

EC Number Inhibitors Comment Organism Structure
1.2.1.105 reactive oxygen species alpha-KGDH can generate reactive oxygen species during its catalytic function, which is regulated by the NADH/NAD+ ratio, formation by alpha-KGDH is attributed to the E3 subunit Mus musculus
1.2.1.105 reactive oxygen species aconitase in the Krebs cycle is more vulnerable than alpha-KGDH to reactive oxygen species, alpha-KGDH can generate reactive oxygen species during its catalytic function, which is regulated by the NADH/NAD+ ratio, as long as alpha-KGDH is functional NADH generation in the Krebs cycle is maintained Rattus norvegicus
1.2.1.105 Zn2+ may play a role in the inhibition of alpha-KGDH during ischemia or reperfusion, is present in elavated concentrations in such cells Rattus norvegicus

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
1.2.1.105 mitochondrion
-
Mus musculus 5739
-
1.2.1.105 mitochondrion
-
Rattus norvegicus 5739
-

Organism

EC Number Organism UniProt Comment Textmining
1.2.1.105 Homo sapiens
-
-
-
1.2.1.105 Mus musculus
-
-
-
1.2.1.105 Rattus norvegicus
-
-
-
1.2.1.105 Sus scrofa
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.2.1.105 brain
-
Mus musculus
-
1.2.1.105 brain
-
Homo sapiens
-
1.2.1.105 forebrain
-
Rattus norvegicus
-
1.2.1.105 heart
-
Sus scrofa
-
1.2.1.105 neuron
-
Rattus norvegicus
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.2.1.105 2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine
-
Rattus norvegicus [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2
-
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Synonyms

EC Number Synonyms Comment Organism
1.2.1.105 alpha-ketoglutarate dehydrogenase
-
Mus musculus
1.2.1.105 alpha-ketoglutarate dehydrogenase
-
Homo sapiens
1.2.1.105 alpha-ketoglutarate dehydrogenase
-
Rattus norvegicus
1.2.1.105 alpha-ketoglutarate dehydrogenase
-
Sus scrofa
1.2.1.105 alpha-KGDH
-
Mus musculus
1.2.1.105 alpha-KGDH
-
Homo sapiens
1.2.1.105 alpha-KGDH
-
Rattus norvegicus
1.2.1.105 alpha-KGDH
-
Sus scrofa

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
1.2.1.105 6.6 7.4
-
Sus scrofa
1.2.1.105 7.2 7.4
-
Mus musculus