LMPD Database

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LMP003836

UniProt Annotations

Entry Information
Gene Namebranched chain keto acid dehydrogenase E1, beta polypeptide
Protein EntryODBB_HUMAN
UniProt IDP21953
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=P21953-1; Sequence=Displayed; Name=2; IsoId=P21953-2; Sequence=VSP_056370, VSP_056371; Note=No experimental confirmation available.;
Catalytic Activity3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2- methylpropanoyl)transferase] S-(2- methylpropanoyl)dihydrolipoyllysine + CO(2).
DiseaseMaple syrup urine disease 1B (MSUD1B) [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).
InteractionP12694:BCKDHA; NbExp=14; IntAct=EBI-1029067, EBI-1029053;
Subcellular LocationMitochondrion matrix.
SubunitHeterotetramer of 2 alpha and 2 beta chains.