Medium-chain specific acyl-CoA dehydrogenase (ACADM) is a homotetramer enzyme of the mitochondrial flavoenzyme family that catalyzes the initial reaction in medium chain fatty acid beta-oxidation (1, 2). ACADM is specific for acyl chain lengths of 4 to 16 (3). It utilizes the electron transfer flavoprotein (ETF) as an electron acceptor that transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (3). Defects in the ACADM gene cause medium-chain acyl-CoA dehydrogenase deficiency (MCADD), a metabolic disorder characterized by fasting hypoglycemia (3). Mutation analysis can diagnose MCADD, which is currently screened in newborns, since its monogene may be modified by genetic variations (4).
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