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Antibody detects endogenous levels of total 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 mitocondrial matrix and uses thiamine pyrophosphate as a cofactor. A congential deficiency in 2-oxoglutarate dehydrogenase activity is believed to lead to hypotonia, metabolic acidosis, and hyperlactatemia.
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Protein Aliases: 2-oxoglutarate dehydrogenase complex component E1; 2-oxoglutarate dehydrogenase E1 component, mitochondrial; 2-oxoglutarate dehydrogenase, mitochondrial; Alpha-ketoglutarate dehydrogenase; Alpha-KGDH-E1; E1o; OGDC-E1; oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide); oxoglutarate decarboxylase; oxoglutarate dehydrogenase (lipoamide); oxoglutarate dehydrogenase (succinyl-transferring); testicular tissue protein Li 131; Thiamine diphosphate (ThDP)-dependent 2-oxoglutarate dehydrogenase
Gene Aliases: 2210403E04Rik; 2210412K19Rik; AA409584; AKGDH; d1401; E1k; Kiaa4192; mKIAA4192; OGDC; OGDH; Ogdhl
UniProt ID: (Human) Q02218, (Mouse) Q60597, (Rat) Q5XI78
Entrez Gene ID: (Human) 4967, (Mouse) 18293, (Rat) 360975
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