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 Dehydrogenase) is a Protein Coding gene. Diseases associated with OGDH include Alpha-Ketoglutarate Dehydrogenase Deficiency and Wernicke-Korsakoff Syndrome. Among its related pathways are Citrate cycle (TCA cycle) and Valproic Acid Pathway, Pharmacodynamics. Gene Ontology (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 OGDHL.
UniProtKB/Swiss-Prot Summary for OGDH Gene
2-oxoglutarate dehydrogenase (E1) component of the 2-oxoglutarate dehydrogenase complex, which mediates the decarboxylation of alpha-ketoglutarate (PubMed:24495017). The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2) (PubMed:24495017). The 2-oxoglutarate dehydrogenase complex is mainly active in the mitochondrion (PubMed:29211711). A fraction of the 2-oxoglutarate dehydrogenase complex also localizes in the nucleus and is required for lysine succinylation of histones: associates with KAT2A on chromatin and provides succinyl-CoA to histone succinyltransferase KAT2A (PubMed:29211711).