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: Lipid Metabolism - synthesis and degradation of ketone bodies; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; EC 18.104.22.168; Carbohydrate Metabolism - butanoate; Transferase
Molecular Function: protein homodimerization activity; 3-oxoacid CoA-transferase activity
Biological Process: response to drug; heart development; response to hormone stimulus; positive regulation of insulin secretion; ketone body metabolic process; cellular lipid metabolic process; response to starvation; response to ethanol; ketone body catabolic process; ketone catabolic process; brain development; response to activity; response to nutrient
SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.