DBT 蛋白
(Dihydrolipoamide Branched Chain Transacylase E2 (DBT))
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].
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Aliases for DBT 蛋白
dihydrolipoamide branched chain transacylase E2 (DBT) 蛋白dihydrolipoamide branched chain transacylase E2 (dbt) 蛋白
dihydrolipoamide branched chain transacylase E2 (CpipJ_CPIJ006326) 蛋白
dihydrolipoamide branched chain transacylase E2 (BDBG_05874) 蛋白
dihydrolipoamide branched chain transacylase E2 (NAEGRDRAFT_78509) 蛋白
dihydrolipoamide branched chain transacylase E2 (VDBG_04820) 蛋白
hypothetical protein (PGTG_17722) 蛋白
dihydrolipoamide branched chain transacylase E2 (Dbt) 蛋白
dihydrolipoamide branched chain transacylase E2 L homeolog (dbt.L) 蛋白
BCATE2 蛋白
BCKAD-E2 蛋白
BCKADE2 蛋白
D3Wsu60e 蛋白
E2 蛋白
E2B 蛋白
E2b 蛋白
im:7147214 蛋白
zgc:103768 蛋白
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