BCKDHA 蛋白
(Branched Chain Keto Acid Dehydrogenase E1, alpha Polypeptide (BCKDHA))
The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Sep 2009].
Recommended next Filters:
10 results
Aliases for BCKDHA 蛋白
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (LOC704978) 蛋白branched chain keto acid dehydrogenase E1, alpha polypeptide (bckdha) 蛋白
branched chain keto acid dehydrogenase E1, alpha polypeptide (BCKDHA) 蛋白
2-oxoisovalerate dehydrogenase alpha subunit (branched-chain alpha-keto acid dehydrogenase E1 component alpha chain) (BCKDH E1-alpha) (BCKDE1A) (BckdhA) 蛋白
branched chain ketoacid dehydrogenase E1, alpha polypeptide (Bckdha) 蛋白
BCKDA 蛋白
BCKDE1A 蛋白
E1a 蛋白
MSU 蛋白
MSUD1 蛋白
OVD1A 蛋白
wu:fd20d04 蛋白
zgc:110049 蛋白
您还需要查找其他产品吗？
You are here:
