Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial (SCOT or OXCT1)

Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial (SCOT/OXCT1), is a mitochondrial matrix enzyme central to ketone body catabolism in extrahepatic tissues. It catalyzes the reversible transfer of coenzyme A from succinyl-CoA to acetoacetate, resulting in the formation of acetoacetyl-CoA and succinate. Acetoacetyl-CoA is subsequently used to produce acetyl-CoA, which enters the citric acid cycle for energy production. SCOT deficiency causes a rare inherited disorder characterized by episodes of severe ketoacidosis. The enzyme is ubiquitously present in extrahepatic tissues but absent from normal liver, enabling tissues to use, but not synthesize, ketone bodies for energy. The human gene is OXCT1. As of the current literature, SCOT is an essential metabolic enzyme but is not a common drug target; its primary clinical significance lies in inherited metabolic disease.

Other names

Succinyl-CoA:3-ketoacid CoA transferase3-Oxoacid CoA-transferase3-oxoacid coenzyme A transferase3-ketoacid CoA-transferaseAcetoacetate succinyl-CoA transferaseAcetoacetyl coenzyme A-succinic thiophoraseSuccinyl coenzyme A-acetoacetyl coenzyme A-transferaseOXCTSCOT1

For drugs hypothetically targeting this enzyme: - Inhibition would prevent ketone body utilization (potentially worsening ketoacidosis) - Activation would increase peripheral ketone utilization (no current drugs specifically with this MoA) - Gene therapy or small molecules could be explored to rescue/restore activity in deficiency (research context)