Tags: Protein.

Peroxisomal acyl-coenzyme A oxidase 1 is an enzyme that in humans is encoded by the ACOX1 gene.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.

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