LMPD Database

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LMP000751

UniProt Annotations

Entry Information
Gene Nameglutaryl-CoA dehydrogenase
Protein EntryGCDH_HUMAN
UniProt IDQ92947
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=Long; IsoId=Q92947-1; Sequence=Displayed; Name=Short; IsoId=Q92947-2; Sequence=VSP_000145;
Biophysicochemical PropertiesKinetic parameters: KM=4.7 uM for glutaryl-CoA (at pH 6.5) ; KM=5.5 uM for glutaryl-CoA (at pH 7.5) ; KM=8.1 uM for glutaryl-CoA (at pH 7.6) ; KM=34.0 uM for glutaryl-CoA (at pH 8.5) ; Note=Release of crotonyl-CoA product from the enzyme is the rate-determining step in its steady-state turnover.;
Catalytic ActivityGlutaryl-CoA + electron-transfer flavoprotein = crotonyl-CoA + CO(2) + reduced electron-transfer flavoprotein.
CofactorName=FAD; Xref=ChEBI
DiseaseGlutaric aciduria 1 (GA1) [MIM
Enzyme RegulationStrongly inhibited by MCPA-CoA, a metabolite of hypoglycin which is present in unripened fruit of the ackee tree.
FunctionCatalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive. {ECO
InteractionP62993:GRB2; NbExp=1; IntAct=EBI-1236978, EBI-401755; P29474:NOS3; NbExp=1; IntAct=EBI-1236978, EBI-1391623; P49768:PSEN1; NbExp=1; IntAct=EBI-1236978, EBI-297277;
PathwayAmino-acid metabolism; lysine degradation.
PathwayAmino-acid metabolism; tryptophan metabolism.
SimilarityBelongs to the acyl-CoA dehydrogenase family.
Subcellular LocationMitochondrion matrix.
SubunitHomotetramer. {ECO
Tissue SpecificityIsoform 1 and isoform 2 are expressed in fibroblasts and liver.