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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]
Enzymes ENZYME proteins Oxidoreductases Predicted intracellular proteins Plasma proteins Citric acid cycle related proteins Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
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GO:0004591 [oxoglutarate dehydrogenase (succinyl-transferring) activity] GO:0005739 [mitochondrion] GO:0005759 [mitochondrial matrix] GO:0006091 [generation of precursor metabolites and energy] GO:0006096 [glycolytic process] GO:0006099 [tricarboxylic acid cycle] GO:0006103 [2-oxoglutarate metabolic process] GO:0006104 [succinyl-CoA metabolic process] GO:0006554 [lysine catabolic process] GO:0006734 [NADH metabolic process] GO:0008152 [metabolic process] GO:0016624 [oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0021695 [cerebellar cortex development] GO:0021756 [striatum development] GO:0021766 [hippocampus development] GO:0021794 [thalamus development] GO:0021860 [pyramidal neuron development] GO:0022028 [tangential migration from the subventricular zone to the olfactory bulb] GO:0030976 [thiamine pyrophosphate binding] GO:0031072 [heat shock protein binding] GO:0031966 [mitochondrial membrane] GO:0034602 [oxoglutarate dehydrogenase (NAD+) activity] GO:0034641 [cellular nitrogen compound metabolic process] GO:0044237 [cellular metabolic process] GO:0044281 [small molecule metabolic process] GO:0045252 [oxoglutarate dehydrogenase complex] GO:0046872 [metal ion binding] GO:0051087 [chaperone binding] GO:0055114 [oxidation-reduction process] GO:0061034 [olfactory bulb mitral cell layer development]
Enzymes ENZYME proteins Oxidoreductases Predicted intracellular proteins Plasma proteins Citric acid cycle related proteins Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
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GO:0004591 [oxoglutarate dehydrogenase (succinyl-transferring) activity] GO:0005739 [mitochondrion] GO:0005759 [mitochondrial matrix] GO:0006091 [generation of precursor metabolites and energy] GO:0006096 [glycolytic process] GO:0006099 [tricarboxylic acid cycle] GO:0006554 [lysine catabolic process] GO:0008152 [metabolic process] GO:0016624 [oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0030976 [thiamine pyrophosphate binding] GO:0031966 [mitochondrial membrane] GO:0034641 [cellular nitrogen compound metabolic process] GO:0044237 [cellular metabolic process] GO:0044281 [small molecule metabolic process] GO:0045252 [oxoglutarate dehydrogenase complex] GO:0046872 [metal ion binding] GO:0055114 [oxidation-reduction process]
Enzymes ENZYME proteins Oxidoreductases Predicted intracellular proteins Plasma proteins Citric acid cycle related proteins Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
Show all
GO:0004591 [oxoglutarate dehydrogenase (succinyl-transferring) activity] GO:0005739 [mitochondrion] GO:0005759 [mitochondrial matrix] GO:0006091 [generation of precursor metabolites and energy] GO:0006096 [glycolytic process] GO:0006099 [tricarboxylic acid cycle] GO:0006554 [lysine catabolic process] GO:0008152 [metabolic process] GO:0016624 [oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor] GO:0030976 [thiamine pyrophosphate binding] GO:0031966 [mitochondrial membrane] GO:0034641 [cellular nitrogen compound metabolic process] GO:0044237 [cellular metabolic process] GO:0044281 [small molecule metabolic process] GO:0045252 [oxoglutarate dehydrogenase complex] GO:0046872 [metal ion binding]