MGP Database

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MGP000295

Ontology/Pathway Information

Entrez Gene ID593
Gene Namebranched chain keto acid dehydrogenase E1, alpha polypeptide
Gene Symbol BCKDHA
SpeciesHomo sapiens
Gene Ontology (GO Annotations)
GO IDSourceTypeDescription
GO:0005947 IDA:HGNCCmitochondrial alpha-ketoglutarate dehydrogenase complex
GO:0005759 TAS:ReactomeCmitochondrial matrix
GO:0005739 IDA:UniProtKBCmitochondrion
GO:0003863 TAS:ProtIncF3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity
GO:0003826 IDA:HGNCFalpha-ketoacid dehydrogenase activity
GO:0016831 TAS:HGNCFcarboxy-lyase activity
GO:0046872 IEA:UniProtKB-KWFmetal ion binding
GO:0009083 IDA:HGNCPbranched-chain amino acid catabolic process
GO:0034641 TAS:ReactomePcellular nitrogen compound metabolic process
GO:0044281 TAS:ReactomePsmall molecule metabolic process
REACTOME Pathway Links
REACTOME Pathway IDDescription
REACT_197Branched-chain amino acid catabolism
REACT_111217Metabolism
REACT_13Metabolism of amino acids and derivatives
SMP Pathway Links
SMP IDDescription
SMP001372-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency
SMP001383-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
SMP005213-hydroxyisobutyric acid dehydrogenase deficiency
SMP005223-hydroxyisobutyric aciduria
SMP002373-Methylcrotonyl Coa Carboxylase Deficiency Type I
SMP001393-Methylglutaconic Aciduria Type I
SMP001403-Methylglutaconic Aciduria Type III
SMP001413-Methylglutaconic Aciduria Type IV
SMP00173Beta-Ketothiolase Deficiency
SMP00523Isobutyryl-coa dehydrogenase deficiency
SMP00524Isovaleric acidemia
SMP00238Isovaleric Aciduria
SMP00199Maple Syrup Urine Disease
SMP00384Methylmalonate Semialdehyde Dehydrogenase Deficiency
SMP00200Methylmalonic Aciduria
SMP00236Propionic Acidemia
SMP00452Threonine and 2-Oxobutanoate Degradation
SMP00032Valine, Leucine and Isoleucine Degradation
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