Aliases for OGDH Gene
External Ids for OGDH Gene
Previous GeneCards Identifiers for OGDH Gene
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]
GeneCards Summary for OGDH Gene
OGDH (Oxoglutarate (Alpha-Ketoglutarate) Dehydrogenase (Lipoamide)) is a Protein Coding gene. Diseases associated with OGDH include alpha-ketoglutarate dehydrogenase deficiency and door syndrome. Among its related pathways are Metabolism and Carbon metabolism. GO annotations related to this gene include chaperone binding and oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor. An important paralog of this gene is DHTKD1.
UniProtKB/Swiss-Prot for OGDH Gene
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3)