HMGCS2 and glutaric aciduria: However, it’s essential to perform acyl-carnitines and urinary organic acids profile in order to achieve a final diagnosis and to differentiate among fatty acid oxidation and ketogenesis defects (Glutaric aciduria type II, carnitine palmitoyltransferase II, carnitine acylcarnitine translocase, deficiency of very-long-chain acyl CoA dehydrogenase , medium-chain acyl-CoA dehydrogenase, 3-OH long-chain acyl CoA dehydrogenase, HMG-CoA lyase and HMG-CoA synthase).