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Literature summary for 1.2.1.105 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

Application Comment Organism
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
medicine plays a role in neurodegenerative diseases Rattus norvegicus
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

Protein Variants Comment Organism
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

Inhibitors Comment Organism Structure
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
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
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

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

Organism

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

Source Tissue

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

Substrates and Products (Substrate)

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

Synonyms

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

pH Optimum

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