Gene:
acyl-Coenzyme A oxidase 1, palmitoyl
Gene ID:
202773
Symbol:
ACOX1
Species:
1000
Description:
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. ACOX1 donates electrons directly to molecular oxygen, thereby producing hydrogen peroxide. Defects in the ACOX1 gene result in pseudoneonatal adrenoleukodystrophy, a disease that is characterized by accumulation of very long chain fatty acids.