Protein Name acyl-CoA oxidase 1
Organism Homo sapiens
Gene ID 51
Gene Symbol


UniProt Q15067 (ACOX1_HUMAN)
Relationships Total Number of functionally related compound(s) : 266
Total Number of Articles : 264

acyl-CoA oxidase 1

Gene Summary

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. [provided by RefSeq, Jul 2008]

  • peroxisomal acyl-coenzyme A oxidase 1
  • acyl-CoA oxidase 1, palmitoyl
  • acyl-CoA oxidase, straight-chain
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