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Official Full Name
dihydrolipoamide branched chain transacylase E2
Background
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.
Synonyms
DBT; dihydrolipoamide branched chain transacylase E2; dihydrolipoamide branched chain transacylase (E2 component of branched chain keto acid dehydrogenase complex; maple syrup urine disease); lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial; E2B; BCATE2; BCKAD E2 subunit; Branched chain acyltransferase, E2 component; Dihydrolipoamide branched chain transacylase; Dihydrolipoyl transacylase; Dihydrolipoyllysine residue (2 methylpropanoyl)transferase; E2; E2 component of branched chain alpha keto acid dehydrogenase complex; Lipoamide acyltransferase component of mitochondrial branched chain alpha keto acid dehydrogenase complex; Mitochondrial branched chain alpha keto acid dehydrogenase transacylase subunit (E2b); BCKADE2; BCKAD-E2; dihydrolipoyllysine-residue (2-methylpropanoyl)transferase; branched-chain alpha-keto acid dehydrogenase complex component E2; E2 component of branched chain alpha-keto acid dehydrogenase complex; mitochondrial branched chain alpha-keto acid dehydrogenase transacylase subunit (E2b); dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex; lipoamide acyltransferase component of mitochondrial branched-chain alpha-keto acid dehydrogenase complex; mitochondrial branched-chain alpha-keto acid dihydrolipoyl acyltransferase; im:7147214; zgc:103768; que

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