LMPD Database

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LMP005481

UniProt Annotations

Entry Information
Gene Namebranched chain keto acid dehydrogenase E1, alpha polypeptide
Protein EntryODBA_HUMAN
UniProt IDP12694
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=P12694-1; Sequence=Displayed; Name=2; IsoId=P12694-2; Sequence=VSP_056156, VSP_056157; 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).
CofactorName=thiamine diphosphate; Xref=ChEBI
DiseaseMaple syrup urine disease 1A (MSUD1A) [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).
InteractionP21953:BCKDHB; NbExp=14; IntAct=EBI-1029053, EBI-1029067;
MiscellaneousBound potassium ions stabilize the protein structure.
Sequence CautionSequence=AAB59549.1; Type=Erroneous initiation; Note=Translation N-terminally extended.; Evidence= ;
SimilarityBelongs to the BCKDHA family.
Subcellular LocationMitochondrion matrix.
SubunitHeterotetramer of alpha and beta chains.