This gene encodes one subunit of the 2-oxoglutarate dehydrogenase complex. This complex catalyzes the overall conversion of 2-oxoglutarate (alpha-ketoglutarate) to succinyl-CoA and CO(2) during the Krebs cycle. The protein is located in the mitocondrial matrix and uses thiamine pyrophosphate as a cofactor. A congential deficiency in 2-oxoglutarate dehydrogenase activity is believed to lead to hypotonia, metabolic acidosis, and hyperlactatemia.
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Protein Aliases: 2-oxoglutarate dehydrogenase complex component E1; 2-oxoglutarate dehydrogenase E1 component, mitochondrial; 2-oxoglutarate dehydrogenase, mitochondrial; Alpha-ketoglutarate dehydrogenase; OGDC-E1; oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide); oxoglutarate decarboxylase; oxoglutarate dehydrogenase (lipoamide); oxoglutarate dehydrogenase (succinyl-transferring); testicular tissue protein Li 131
Gene Aliases: AKGDH; E1k; OGDC; OGDH; Ogdhl