Molecular Classification
Enzyme, Oxidoreductase, Dehydrogenase complex
Other Names
KGDHC, 2-oxoglutarate dehydrogenase complex, α-ketoglutarate dehydrogenase
Disease Roles
Alpha-Ketoglutarate Dehydrogenase Complex Overview
Alpha-ketoglutarate dehydrogenase complex (α-KGDH or KGDHC) is a large mitochondrial enzyme complex that catalyzes the oxidative decarboxylation of alpha-ketoglutarate to succinyl-CoA and CO₂ in the citric acid (Krebs) cycle. This reaction also produces NADH, which directly supplies electrons to the respiratory chain for ATP production. Impaired function of this enzyme has been found to be characteristic for several neurodegenerative diseases.
Mechanism of Action
N/A
Biological Functions
Citric acid cycle
Oxidative decarboxylation
ATP production
Redox homeostasis
Cellular respiration
Disease Associations
Neurodegenerative disease
Alzheimer's disease
Metabolic encephalopathy
Alpha-ketoglutarate dehydrogenase deficiency syndrome
Safety Considerations
- Impaired energy metabolism
- Reactive oxygen species (ROS) production
- Neurological dysfunction