Succinyl-CoA:3-oxoacid CoA transferase 1 (OXCT1)

Succinyl-CoA:3-oxoacid CoA transferase 1 (OXCT1) is a mitochondrial enzyme that catalyzes the reversible transfer of coenzyme A from succinyl-CoA to acetoacetate, forming acetoacetyl-CoA. This reaction constitutes the first, rate-limiting step in the extrahepatic catabolism of ketone bodies (ketolysis), enabling tissues such as heart, brain, and kidney to utilize ketones as energy sources during fasting, illness, or exercise. OXCT1 is absent from the liver, distinguishing tissue-specific roles in ketogenesis and ketolysis. Mutations in OXCT1 can result in succinyl-CoA:3-oxoacid CoA transferase deficiency, a metabolic disorder characterized by episodes of severe ketoacidosis. OXCT1 is also implicated in diabetes—where its activity is reduced in pancreatic islets—and in tumor metabolism, where its function may provide proliferating cancer cells with an alternative energy source

Other names

3-oxoacid CoA transferase 13-oxoacid-CoA transferase 1OXCTSCOTSCOT1_HUMANsomatic-type succinyl CoA:3-oxoacid CoA-transferasesomatic-type succinyl-CoA:3-oxoacid-CoA-transferasesuccinyl CoA:3-oxoacid CoA transferasesuccinyl-CoA:3-ketoacid-CoA transferasesuccinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial

Inhibition may impair ketone body utilization and energy metabolism; potential for tumor metabolic targeting