MitoProteome Database

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MT000075

Gene Information

Entrez Gene ID594
Gene Namebranched chain keto acid dehydrogenase E1, beta polypeptide
Gene SymbolBCKDHB
SpeciesHomo sapiens
Gene Ontology (GO Annotations)
GO IDSourceTypeDescription
GO:0005947 IMP:HGNCCmitochondrial alpha-ketoglutarate dehydrogenase complex
GO:0005759 TAS:ReactomeCmitochondrial matrix
GO:0005739 IMP:HGNCCmitochondrion
GO:0003863 TAS:ProtIncF3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity
GO:0003826 IEA:EnsemblFalpha-ketoacid dehydrogenase activity
GO:0016831 TAS:HGNCFcarboxy-lyase activity
GO:0009083 IMP:HGNCPbranched-chain amino acid catabolic process
GO:0034641 TAS:ReactomePcellular nitrogen compound metabolic process
GO:0051591 IEA:EnsemblPresponse to cAMP
GO:0051384 IEA:EnsemblPresponse to glucocorticoid
GO:0007584 IEA:EnsemblPresponse to nutrient
GO:0044281 TAS:ReactomePsmall molecule metabolic process
KEGG Pathway Links
KEGG Pathway IDDescription
hsa_M00036Leucine degradation, leucine => acetoacetate + acetyl-CoA
M00036Leucine degradation, leucine => acetoacetate + acetyl-CoA
hsa01100Metabolic pathways
hsa00280Valine, leucine and isoleucine degradation
ko00280Valine, leucine and isoleucine degradation
BIOCYC Pathway Links
BIOCYC Pathway IDDescription
PWY-51302-oxobutanoate degradation I
PWY-51302-oxobutanoate degradation I
PWY-50462-oxoisovalerate decarboxylation to isobutanoyl-CoA
PWY-50462-oxoisovalerate decarboxylation to isobutanoyl-CoA
ILEUDEG-PWYisoleucine degradation I
ILEUDEG-PWYisoleucine degradation I
PWY-5328superpathway of methionine degradation
PWY-5328superpathway of methionine salvage and degradation
VALDEG-PWYvaline degradation I
VALDEG-PWYvaline degradation I
REACTOME Pathway Links
REACTOME Pathway IDDescription
REACT_197Branched-chain amino acid catabolism
REACT_111217Metabolism
REACT_13Metabolism of amino acids and derivatives