DBT Products

Description

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]

Bioinformatics

Entrez	1629 Human
Uniprot	AAH16675.1 Human NM_001918 Human P11182 Human
Product By Gene ID	1629
Alternate Names	BCATE2BCKAD E2 subunit BCKADE2 BCKAD-E2 branched chain acyltransferase, E2 component Branched-chain alpha-keto acid dehydrogenase complex component E2 Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto aciddehydrogenase complex dihydrolipoamide branched chain transacylase (E2 component of branched chainketo acid dehydrogenase complex; maple syrup urine disease) dihydrolipoamide branched chain transacylase E2 Dihydrolipoamide branched chain transacylase dihydrolipoyl transacylase Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase E2 component of branched chain alpha-keto acid dehydrogenase complex E2 E2B EC 2.3.1.168 lipoamide acyltransferase component of branched-chain alpha-keto aciddehydrogenase complex, mitochondrial lipoamide acyltransferase component of mitochondrial branched-chain alpha-ketoacid dehydrogenase complex MGC9061 mitochondrial branched chain alpha-keto acid dehydrogenase transacylase subunit(E2b)

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Stem Cell Markers