phytanoyl-CoA + 2-oxoglutarate + O2 => 2-hydroxyphytanoyl-CoA + succinate + CO2 Authored: D'Eustachio, P, 2009-01-10 20:36:15 EC Number: 1.14.11.18 Edited: D'Eustachio, P, 2009-01-10 20:36:15 Peroxisomal phytanoyl-CoA dioxygenase catalyzes the reaction of phytanoyl-CoA, 2-oxoglutarate, and O2 to form 2-hydroxyphytanoyl-CoA, succinate, and CO2. The mature form of the enzyme lacks the first 30 amino acid residues of the full-length polypeptide and is complexed with Fe++. Mutations in this enzyme are the commonest cause of Refsum disease (Mukherji et al. 2001; McDonough et al. 2005). Pubmed11555634 Pubmed16186124 Reactome Database ID Release 43389639 Reactome, http://www.reactome.org ReactomeREACT_17021 Reviewed: Jassal, B, 2009-02-27 15:21:18