Recombinant Human Branched Chain keto Acid Dehydrogenase E1, Alpha
供应商
ProSpec
产品货号
ENZ-090
产品报价
¥询价/1ug ¥50ug ¥5ug
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产品新闻
背景资料
Branched-chain ?-keto acid dehydrogenase E1 component ? chain(BCKDHA) is a member of the BCKDHA family. The BCKD(branched-chain alpha-keto acid dehydrogenase) complex is an inner mitochondrial enzyme complex which catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. This complex consists of 3 catalytic components: a heterotetrameric(alpha2-beta2) branched-chain alpha-keto acid decarboxylase(E1), a dihydrolipoyl transacylase(E2), and a dihydrolipoamide dehydrogenase(E3). Mutations in the BCKDHA gene result in maple syrup urine disease, type IA.