<unk>  is an inborn error of ketone body utilization.<unk> -CoA:3-oxoacid<unk><unk>  catalyzes the transfer of coenzyme A from<unk> -coenzyme A to acetoacetate. It can be caused by mutation in the OXCT1 gene. 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.
Succinyl-CoA:3-oxoacid CoA transferase deficiency
3-Hydroxyisobutyryl-CoA deacylase deficiency
2-Methylbutyryl-CoA dehydrogenase deficiency
Malonyl-CoA decarboxylase deficiency
3-Methylcrotonyl-CoA carboxylase deficiency
Pyruvate kinase deficiency
3-hydroxyacyl-coenzyme A dehydrogenase deficiency
Glutaminase deficiency