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OGDH

Official Full Name
oxoglutarate dehydrogenase
Organism
Homo sapiens
GeneID
4967
Background
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 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.[provided by RefSeq, Sep 2009]
Synonyms
E1k; E1o; KGD1; OGDC; AKGDH; OGDH2; OGDHD; HsOGDH; OGDH-E1;

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