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The residual activity of the CPT II enzyme is very low in neonatal and infantile forms, resulting in abnormal LCFA profiles, notably with high levels of C16 and C18OH ( Clinical and metabolic features In 1973, DiMauro and DiMauro reported the first case of an adult-onset form of CPT II deficiency ( Carnitine plays a crucial role in the entry of LCFA via carnitine shuttle into the mitochondrial matrix for -oxidation and energy production in the form of adenosine triphosphate