Mitochondrial acetoacetyl-CoA thiolase (T2; EC 2.3.1.9) deficiency is an autosomal recessive disorder that affects the catabolism of isoleucine and ketone bodies (1,2). This disorder, commonly known ...
Acetoacetate and 3-hydroxybutyrate are slightly acidic biomolecules that, together with acetone, are called ketone bodies and serve as the major circulating energy source during fasting. Ketone bodies ...