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Mitochondrial fatty acid beta-oxidation is one of the main energy-producing metabolic pathways in eukaryotes. Acyl-CoA dehydrogenases (ACADs; EC 1.3.99.13) are mitochondrial enzymes that catalyze the initial rate-limiting step in the beta-oxidation of fatty acyl-CoA. ACAD9 belongs to a group of ACADs that act on fatty acids containing 14 to 20 carbons (Zhang et al., 2002 [PubMed 12359260]).[supplied by OMIM]