Acyl Coenzyme A Oxidase 2, Branched (ACOX2)

ACOX2 is 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 mental retardation, and death in children.Peroxisomes in human liver contain 2 distinct acyl-CoA oxidases with different substrate specificities: palmitoyl-CoA oxidase, oxidizing very long straight-chain fatty acids and eicosanoids, and a branched-chain acyl-CoA oxidase, involved in the degradation of long branched fatty acids and bile acid intermediates. The accumulation of branched fatty acids and bile acid intermediates leads to severe mental retardation and death of affected children.