acyl-CoA oxidase 2
The information on this page was automatically extracted from online scientific databases.
From NCBI Gene:
The product of this gene 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 mental retardation, and death in children. [provided by RefSeq, Mar 2009]
Oxidizes the CoA esters of the bile acid intermediates di- and tri-hydroxycholestanoic acids.