Succinyl-CoA:3-oxoacid CoA transferase deficiency

Succinyl-CoA:3-oxoacid CoA transferase deficiency is an inborn error of ketone body utilization. Succinyl-CoA:3-oxoacid CoA transferase catalyzes the transfer of coenzyme A from succinyl-coenzyme A to acetoacetate. It can be caused by mutation in the OXCT1 gene.

Succinyl-CoA:3-oxoacid CoA transferase deficiency
Other names	SCOT deficiency
Succinyl-CoA:3-oxoacid CoA transferase deficiency is inherited via autosomal recessive manner

First described in 1972, more than 30 people have been reported in the medical literature with this inborn error of metabolism. They experience attacks of ketoacidosis during illness, and even when well may have elevated levels of ketone bodies in blood and urine (ketonemia and ketonuria, respectively). Not all people with SCOT deficiency have persistent ketonemia and ketonuria, particularly those with milder defects of enzyme activity.[1]