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OXCT1 Key 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. Defects in OXCT1 are a cause of succinyl-CoA-3-ketoacid- CoA transferase deficiency (SCOTD). A disorder of ketone body metabolism, characterized by episodic ketoacidosis. Patients are usually asymptomatic between episodes. Belongs to the 3-oxoacid CoA-transferase family. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - butanoate; EC; Lipid Metabolism - synthesis and degradation of ketone bodies; Mitochondrial; Transferase
Chromosomal Location of Human Ortholog: 5p13.1
Cellular Component: mitochondrial matrix; mitochondrion; nucleoplasm
Molecular Function: 3-oxoacid CoA-transferase activity
Biological Process: ketone body catabolic process; ketone body metabolic process
Disease: Succinyl-coa:3-oxoacid Coa Transferase Deficiency
Reference #:  P55809 (UniProtKB)
Alt. Names/Synonyms: 3-oxoacid CoA transferase 1; 3-oxoacid-CoA transferase 1; OXCT; OXCT1; SCOT; SCOT-s; SCOT1; somatic-type succinyl CoA:3-oxoacid CoA-transferase; Somatic-type succinyl-CoA:3-oxoacid-CoA-transferase; succinyl CoA:3-oxoacid CoA transferase; succinyl-CoA:3-ketoacid-CoA transferase; Succinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial
Gene Symbols: OXCT1
Molecular weight: 56,158 Da
Basal Isoelectric point: 7.13  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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