重组支链α-酮酸脱氢酶E2蛋白

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重组支链α-酮酸脱氢酶E2蛋白

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Recombinant DBT
Recombinant dihydrolipoamide branched chain transacylase E2 protein

基因名：

DBT

产品别名：

BCATE2； BCKAD-E2； BCKADE2； BCKDH-E2； BCOADC-E2； E2； E2B； DBT； dihydrolipoamide branched chain transacylase E2； dihydrolipoamide branched chain transacylase E2； lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial； 52 kDa mitochondrial autoantigen of primary biliary cirrhosis； BCKAD E2 subunit； E2 component of branched chain alpha-keto acid dehydrogenase complex； branched chain 2-oxo-acid dehydrogenase complex component E2； branched chain acyltransferase, E2 component； branched-chain alpha-keto acid dehydrogenase complex component E2； dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex； dihydrolipoyl transacylase； dihydrolipoyllysine-residue (2-methylpropanoyl)transferase； lipoamide acyltransferase component of mitochondrial branched-chain alpha-keto acid dehydrogenase complex； mitochondrial branched chain alpha-keto acid dehydrogenase transacylase subunit (E2b)；支链α-酮酸脱氢酶E2；

背景信息：

The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined. [provided by RefSeq, Jul 2008]