ACOX2 (Acyl-CoA Oxidase 2) is a Protein Coding gene. Diseases associated with ACOX2 include Bile Acid Synthesis Defect, Congenital, 6 and Congenital Bile Acid Synthesis Defect. Among its related pathways are Metabolism and Synthesis of bile acids and bile salts. The product of ACOX2 belongs to the acyl-CoA oxidase family. It encodes the branched-chain acyl-CoA oxidase which is involved in the degradation of long branched fatty acids and bile acid intermediates in peroxisomes. Deficiency of this enzyme results in the accumulation of branched fatty acids and bile acid intermediates, and may lead to Zellweger syndrome, severe cognitive disability, and death in children.