EC Number   |
Natural Substrates |
|---|
    1.2.1.105 | 2-oxoglutarate + CoA + NAD+ |
- |
| 1.2.1.105 | 2-oxoglutarate + lipoamide |
- |
| 1.2.1.105 | 2-oxoglutarate + lipoamide |
catalyzes a rate limiting step of the TCA cycle |
| 1.2.1.105 | 2-oxoglutarate + lipoamide |
the 2-oxoglutarate dehydrogenase complex catalyzes a critical step in the Krebs tricarboxylic acid cycle, which is also a step in the metabolism of the potentially excitotoxic neurotransmitter glutamate. Deficiencies of the 2-oxoglutarate dehydrogenase complex are likely to impair energy metabolism and therfore brain function, and lead to manifestations of brain disease. Neurons that are enriched in the 2-oxoglutarate dehydrogenase complex may be selectively vulnerable in Alzheimers disease. Variations in 2-oxoglutarate dehydrogenase complex that are not deleterious during reproductive life become deleterious with aging, perhaps by predisposing this mitochondrial metabolon to oxidative damage |
| 1.2.1.105 | 2-oxoglutarate + lipoamide |
addition of glutamate stimulates the synthesis of the 2-oxoglutarate dehydrogenase complex |
| 1.2.1.105 | 2-oxoglutarate + lipoamide |
enzyme of the TCA cycle |
| 1.2.1.105 | 2-oxoglutarate + lipoamide |
reductions in enzyme activity occurs in a number of neurodegenerative disorders including Alzheimers disease. The reduction in 2-oxoglutarate dehydrogenase complex activity can be linked to several aspects of brain dysfunction and neuropathology in a number of neurodegenerative diseases |
| 1.2.1.105 | 2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine |
- |
| 1.2.1.105 | 2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine |
enzyme E2 is a component of the 2-oxoglutarate dehydrogenase multienzyme complex, rate-limiting enzyme in mitochondrial Krebs cycle |
| 1.2.1.105 | 2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine |
key step, probably rate-limiting, in the tricarboxylic acid cycle, physiologic function and regulation |
