ACADL

acyl-CoA dehydrogenase, long chain

Long-chain acyl-coA dehydrogenase (ACADL), also abbreviated as LCAD, is a mitochondrial metrix enzyme involved in catalyzing the first reaction in the mitochondrial beta-oxidation cycles for fatty acids. Two other acyl-coA dehydrogenases are also involved in fatty acid oxidation, medium chain and short chain ALDs. However, of these ACADL catalyzes the first and critical step in this process. ACADL is a homotetrameic enzyme of 45-kD. It is synthesized in the cytoplasm a a precursor then processed and targeted to the mitochondria. Genetic deficiency of ACADL is associated with severe hypoglycemia, skeletal muscle weakness, hepatomegaly and cardiomegaly, which can be fatal.

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