PathwayStep1400 PathwayStep1401 PathwayStep1402 PathwayStep1403 PathwayStep1404 PathwayStep1405 PathwayStep1406 phytanoyl-CoA + 2-oxoglutarate + O2 => 2-hydroxyphytanoyl-CoA + succinate + CO2 phytanate + CoA-SH + ATP => phytanoyl-CoA + AMP + pyrophosphate PMP34-mediated exchange of cytosolic ATP for peroxisomal AMP 2-hydroxyphytanoyl-CoA => pristanal + formyl-CoA formyl-CoA + H2O => formate + CoASH pristanal + NAD+ => pristanate + NADH + H+ pristanate + CoA-SH + ATP => pristanoyl-CoA + AMP + pyrophosphate Alpha-oxidation of phytanate Authored: D'Eustachio, P, 2009-01-10 20:36:15 Edited: D'Eustachio, P, 2009-01-10 20:36:15 GENE ONTOLOGYGO:0001561 Phytanic acid arises through ruminant metabolism of chlorophyll and enters the human diet as a constituent of dairy products (Baxter 1968). It can act as an agonist for PPAR and other nuclear hormone receptors, but its normal role in human physiology, if any, is unclear. It is catabolized via a five-step alpha-oxidation reaction sequence that yields pristanoyl-CoA, which is turn is a substrate for beta-oxidation. These reactions take place in the peroxisomal matrix and their failure is associated with Refsum disease (Wanders et al. 2003). Pubmed12633678 Pubmed4177872 Reactome Database ID Release 43389599 Reactome, http://www.reactome.org ReactomeREACT_17003 Reviewed: Jassal, B, 2009-02-27 15:21:18