重组酮戊二酸脱氢酶(OGDH)蛋白

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重组酮戊二酸脱氢酶(OGDH)蛋白

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重组酮戊二酸脱氢酶OGDH蛋白
Recombinant OGDH

基因名：

OGDH

产品别名：

AKGDH； E1k； E1o； KGD1； OGDC； OGDH-E1； OGDH2； OGDHD； OGDH； oxoglutarate dehydrogenase； oxoglutarate dehydrogenase； 2-oxoglutarate dehydrogenase complex component E1； 2-oxoglutarate dehydrogenase, mitochondrial； OGDC-E1； alpha-KGDH-E1； oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)； oxoglutarate decarboxylase； oxoglutarate dehydrogenase (succinyl-transferring)； testicular tissue protein Li 131； thiamine diphosphate (ThDP)-dependent 2-oxoglutarate dehydrogenase；酮戊二酸脱氢酶(OGDH)；酮戊二酸脱氢酶OGDH；

背景信息：

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]