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OGDH is 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 mitochondrial matrix and uses thiamine pyrophosphate as a cofactor. A congenital deficiency in 2-oxoglutarate dehydrogenase activity is believed to lead to hypotonia, metabolic acidosis, and hyperlactatemia. Alternative splicing results in multiple transcript variants encoding distinct isoforms.
Gene Name: | oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide) |
Synonyms: | OGDH, AKGDH, E1k, Oxoglutarate decarboxylase, OGDC, OGDC-E1 |
Target Sequences: | NM_002541 NP_002532.2 Q02218 |
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