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During mitochondrial oxidation the first oxidation step, catalyzed by various acyl-CoA dehydrogenases, results in the reduced electron carrier FADH 2 that then delivers its electrons directly to the electron transport chain for synthesis of ATP

Consequently, patience during reconstitution protects the integrity of all four compounds simultaneously

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Clinical Features of Primary Carnitine Deficiency The typical metabolic presentation in patients with primary carnitine deficiency occurs most frequently before 2 years of age

NADPH has a characteristic absorption peak at 340 nm, while NADP+ does not