Q15067

ACOX1(Acyl-coenzyme A oxidase 1) is a crucial 660 amino acid protein localized in the peroxisome, where ACOX1 plays a vital role in lipid metabolism. Specifically, ACOX1 catalyzes the desaturation of very long chain acyl-CoA substrates into 2-trans-enoyl-CoA, a reaction that is essential for the breakdown of fatty acids and involves the use of FAD as a cofactor. This enzymatic activity not only facilitates energy production but also contributes to the regulation of cellular lipid levels, which is critical for maintaining cellular homeostasis. Defects in ACOX1 can lead to pseudoneonatal adrenoleukodystrophy (pseudo-NALD), a serious condition characterized by neurological impairments and metabolic dysfunctions, underscoring ACOX1′s significance in human health.