3-ketopristanoyl-CoA + CoASH => 4,8,12-trimethyltridecanoyl-CoA + propionyl-CoA Authored: D'Eustachio, P, 2009-03-16 18:50:14 EC Number: 2.3.1.154 Edited: D'Eustachio, P, 2009-03-18 13:33:40 Peroxisomal SCPx (Non-specific lipid transfer protein; SCP2) catalyzes the reaction of 3-ketopristanoyl-CoA and CoASH to form 4,8,12-trimethyltridecanoyl-CoA and propionyl-CoA. Both intact SCPx and an SCPx fragment corresponding to approximately the 430 aminoterminal residues of the protein are catalytically active in vitro; the latter form may predominate in vivo. Consistent with the role of SCPx in the beta-oxidation of branched-chain fatty acids in vitro, mutations in the protein are associated with elevated levels of pristanic acid in the blood in vivo and the development of neurological defects (Ferdinandusse et al. 2000, 2006). Pubmed10706581 Pubmed16685654 Reactome Database ID Release 43390224 Reactome, http://www.reactome.org ReactomeREACT_17019 Reviewed: Jassal, B, 2009-02-27 15:21:18