LMPD Database

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LMP001815

UniProt Annotations

Entry Information
Gene Name3-oxoacid CoA transferase 1
Protein EntrySCOT1_HUMAN
UniProt IDP55809
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=P55809-1; Sequence=Displayed; Name=2; IsoId=P55809-2; Sequence=VSP_056310; Note=No experimental confirmation available.;
Catalytic ActivitySuccinyl-CoA + a 3-oxo acid = succinate + a 3- oxoacyl-CoA.
DiseaseSuccinyl-CoA-3-ketoacid-CoA transferase deficiency (SCOTD) [MIM
FunctionKey enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate.
PathwayKetone metabolism; succinyl-CoA degradation; acetoacetyl- CoA from succinyl-CoA
SimilarityBelongs to the 3-oxoacid CoA-transferase family.
Subcellular LocationMitochondrion matrix.
SubunitHomodimer.
Tissue SpecificityAbundant in heart, followed in order by kidney, brain, and muscle, whereas in liver it is undetectable; also detectable in leukocytes and fibroblasts.