ACADM

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ACADM

A gene on chromosome 1p31, which encodes a medium-chain-specific (C4 to C12 straight chain) acyl-Coenzyme A dehydrogenase, which catalyses the initial step of the mitochondrial fatty acid beta-oxidation pathway.

Molecular pathology
ACADM defects cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterised by hepatic dysfunction, fasting hypoglycemia and encephalopathy, which causes death in infancy.