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MGP002179

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. {ECO:0000255|PROSITE-ProRule:PRU10034}.
DiseaseSuccinyl-CoA-3-ketoacid-CoA transferase deficiency (SCOTD) [MIM:245050]: A disorder of ketone body metabolism, characterized by episodic ketoacidosis. Patients are usually asymptomatic between episodes. {ECO:0000269|PubMed:10964512, ECO:0000269|PubMed:21296660, ECO:0000269|PubMed:9671268}. Note=The disease is caused by mutations affecting the gene represented in this entry.
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: step 1/1.
SimilarityBelongs to the 3-oxoacid CoA-transferase family. {ECO:0000305}.
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.
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