The protein encoded by this gene is the first enzyme of the fatty acid beta-oxidation pathway, which catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. It donates electrons directly to molecular oxygen, thereby producing hydrogen peroxide. Defects in this gene result in pseudoneonatal adrenoleukodystrophy, a disease that is characterized by accumulation of very long chain fatty acids. Alternatively spliced transcript variants encoding different isoforms have been identified.
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Protein Aliases: acyl-CoA oxidase 1, palmitoyl; acyl-CoA oxidase, straight-chain; acyl-Coenzyme A oxidase 1, palmitoyl; AOX; Palmitoyl-CoA oxidase; Peroxisomal acyl-CoA oxidase 1, A chain; Peroxisomal acyl-CoA oxidase 1, B chain; Peroxisomal acyl-CoA oxidase 1, C chain; Peroxisomal acyl-coenzyme A oxidase 1; peroxisomal fatty acyl-CoA oxidase; peroxisomal fatty acyl-coenzyme A oxidase; peroxisomal straight-chain Acyl-CoA oxidase; SCOX; Straight-chain acyl-CoA oxidase
Gene Aliases: ACOX; ACOX1; AOX; D130055E20Rik; PALMCOX; Paox; RATACOA1; SCOX