MitoSciences: Alpha-ketoglutarate dehydrogenase (OGDH)

UniProt Number:	Q02218, P36957, P09622
Alternate Names:	Oxoglutarate dehydrogenase, 2-oxoglutarate dehydrogenase, 2-oxoglutarate dehydrogenase complex, OGDC
Structure and Function:	OGDH is a large protein complex consisting of 3 different components. These are oxoglutarate dehydrogenase (gene OGDH), which contains thiamine pyrophosphate as a prosthetic group, dihydrolipoyl succinyltransferase (DLST) which contains lipoic acid, and dihydrolipoyl dehydrogenase (DLD). The complex which is located in the mitochondrial matrix catalyzes the decarboxylation of alpha ketoglutarate to produce succinyl CoA with reduction of NAD+ to generate NADH. OGDH is a key component of the Krebs Cycle and is under tight control by product and substrate, and through availability of alpha ketoglutarate links the Krebs cycle to amino acid metabolism and formation of glutamate.
Disease Associations:	OGDH is a well-characterized auto-antigen in primary biliary cirrhosis. The activity of the enzyme is much lower in Alzheimer's disease, and there are reports of the enzyme's susceptibility to modification by oxidative stress.