peroxisomal acyl-coenzyme A oxidase 1; AOX; palmitoyl-CoA oxidase; straight-chain acyl-CoA oxidase; SCOX (ACOX1)
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Function
- catalyzes desaturation of very long chain acyl-CoAs to 2-trans-enoyl-CoAs
- lipid metabolism; peroxisomal fatty acid beta-oxidation
acyl-CoA + O2 = trans-2,3-dehydroacyl-CoA + H2O2
Structure
belongs to the acyl-CoA oxidase family
Compartment
Alternative splicing
named isoforms=2
Pathology
- defects in ACOX1 are the cause of adrenoleukodystrophy pseudoneonatal