OGDH blocking peptide
OGDH Antibody (C-term) Blocking peptide
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
OGDH: 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). Belongs to the alpha-ketoglutarate dehydrogenase family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 1.2.4.2; Mitochondrial; Amino Acid Metabolism - lysine degradation; Amino Acid Metabolism - tryptophan; Carbohydrate Metabolism - citrate (TCA) cycle; Oxidoreductase
Chromosomal Location of Human Ortholog: 7p14-p13
Cellular Component: cytosol; mitochondrial matrix; mitochondrial membrane; mitochondrion; oxoglutarate dehydrogenase complex
Molecular Function: oxoglutarate dehydrogenase (succinyl-transferring) activity
Biological Process: generation of precursor metabolites and energy; glyoxylate metabolic process; lysine catabolic process; tricarboxylic acid cycle
Disease: Alpha-ketoglutarate Dehydrogenase Deficiency