LMPD Database

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LMP001763

UniProt Annotations

Entry Information
Gene Namedihydrolipoamide branched chain transacylase E2
Protein EntryODB2_HUMAN
UniProt IDP11182
SpeciesHuman
Comments
Comment typeDescription
Catalytic Activity2-methylpropanoyl-CoA + enzyme N(6)- (dihydrolipoyl)lysine = CoA + enzyme N(6)-(S-(2- methylpropanoyl)dihydrolipoyl)lysine.
CofactorName=(R)-lipoate; Xref=ChEBI
DiseaseMaple syrup urine disease 2 (MSUD2) [MIM
FunctionThe branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component.
Sequence CautionSequence=AAA35589.1; Type=Erroneous initiation; Evidence= ; Sequence=AAA64512.1; Type=Erroneous initiation; Evidence= ;
SimilarityBelongs to the 2-oxoacid dehydrogenase family.
SimilarityContains 1 lipoyl-binding domain.
Subcellular LocationMitochondrion matrix.
SubunitForms a 24-polypeptide structural core with octahedral symmetry.