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].
Baumgart, Vanhooren, Fransen, Marynen, Puype, Vandekerckhove, Leunissen, Fahimi, Mannaerts, van Veldhoven et al.: "Molecular characterization of the human peroxisomal branched-chain acyl-CoA oxidase: cDNA cloning, chromosomal assignment, tissue distribution, and evidence for the absence of the protein in ..." in: Proceedings of the National Academy of Sciences of the United States of America, Vol. 93, Issue 24, pp. 13748-53, (1997) (PubMed).