LMPD Database

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LMP000673

UniProt Annotations

Entry Information
Gene Namebranched chain ketoacid dehydrogenase kinase
Protein EntryBCKD_HUMAN
UniProt IDO14874
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=3; Name=1; IsoId=O14874-1; Sequence=Displayed; Name=2; IsoId=O14874-2; Sequence=VSP_054604, VSP_054605; Note=No experimental confirmation available.; Name=3; IsoId=O14874-3; Sequence=VSP_054605; Note=No experimental confirmation available.;
Catalytic ActivityATP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] = ADP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphate.
DiseaseBranched-chain ketoacid dehydrogenase kinase deficiency (BCKDKD) [MIM
FunctionCatalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex (By similarity).
InteractionQ9WMX2:- (xeno); NbExp=2; IntAct=EBI-1046765, EBI-6863741; P40763:STAT3; NbExp=2; IntAct=EBI-1046765, EBI-518675;
PtmAutophosphorylated.
SimilarityBelongs to the PDK/BCKDK protein kinase family.
SimilarityContains 1 histidine kinase domain.
Subcellular LocationMitochondrion matrix.
SubunitMonomer.
Tissue SpecificityUbiquitous.
Web ResourceName=Protein Spotlight; Note=The silence within - Issue 147 of March 2013; URL="http://web.expasy.org/spotlight/back_issues/147";