OGDH

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]

Description

oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)

Protein family

Citric acid cycle related proteins, Enzymes, Mapped to UniProt SWISS-PROT, Mitochondrial proteins, Plasma proteins, Predicted intracellular proteins, Protein evidence (Ezkurdia et al 2014), Protein evidence (Kim et al 2014)

Chromosome

7:p13 (44606572 - 44709066)

Ensembl ID

ENSG00000105953  (ver. 84.38)

Orthologs

Mouse Ogdh: ENSMUSG00000020456

UniProt

Q02218

neXtProt

NX_Q02218

Human splice variants
Mouse splice variants