Inhibitors | Comment | Organism | Structure |
---|---|---|---|
2-oxo-3-methyl-n-valeric acid | inhibition of the 2-oxoglutarate dehydrogenase enzyme complex in vivo and in situ, after 40 min 25% inhibition at 10 mM, 46% at 20 mM, after 80 min 58% inhibition at 10 mM, 80% at 20 mM, inhibition does not affect the mitochondrial membrane potential | Homo sapiens | |
additional information | inhibition of the alpha-ketoglutarate dehydrogenase complex alters mitochondrial function and cellular calcium regulation | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Homo sapiens | 5739 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine | Homo sapiens | key step, probably rate-limiting, in the tricarboxylic acid cycle, physiologic function and regulation | [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2 | - |
ir | |
additional information | Homo sapiens | reduced enzyme activity in vivo does not lead to the Ca2+ abnormalities observed in patients suffering brain disorders and abnormal mitochondrial function, or Alzheimer's disease, but the release of cytochrome c together with reduced enzyme activity activates other pathways including cell death cascades, enzyme inhibition alters Ca2+ homeostasis and increases cytosolic accumulation of cytochrome c | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
enzyme is part of the alpha-ketoglutarate dehydrogenase complex KGDHC | - |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
brain | - |
Homo sapiens | - |
fibroblast | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine | - |
Homo sapiens | [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2 | - |
ir | |
2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine | key step, probably rate-limiting, in the tricarboxylic acid cycle, physiologic function and regulation | Homo sapiens | [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2 | - |
ir | |
additional information | reduced enzyme activity in vivo does not lead to the Ca2+ abnormalities observed in patients suffering brain disorders and abnormal mitochondrial function, or Alzheimer's disease, but the release of cytochrome c together with reduced enzyme activity activates other pathways including cell death cascades, enzyme inhibition alters Ca2+ homeostasis and increases cytosolic accumulation of cytochrome c | Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
More | enzyme is part of the alpha-ketoglutarate dehydrogenase complex KGDHC | Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
NAD+ | - |
Homo sapiens |