PathwayStep1415 PathwayStep1416 PathwayStep1413 PathwayStep1414 PathwayStep1411 PathwayStep1412 PathwayStep1410 PathwayStep1417 PathwayStep1418 PathwayStep1408 PathwayStep1409 3-ketopristanoyl-CoA + CoASH => 4,8,12-trimethyltridecanoyl-CoA + propionyl-CoA 3-hydroxypristanoyl-CoA + NAD+ => 3-ketoxypristanoyl-CoA + NADH + H+ Isomerization of (2R)-pristanoyl-CoA to (2S)-pristanoyl-CoA (2S)-pristanoyl-CoA + O2 => trans-2,3-dehydropristanoyl-CoA + H2O2 (ACOX2) (2S)-pristanoyl-CoA + O2 => trans-2,3-dehydropristanoyl-CoA + H2O2 (ACOX3) trans-2,3-dehydropristanoyl-CoA + H2O => 3-hydroxypristanoyl-CoA acetyl-CoA + H2O => acetate + CoASH propionyl-CoA + carnitine => propionylcarnitine + CoASH 4,8-dimethylnonanoyl-CoA + carnitine => 4,8-dimethylnonanoylcarnitine + CoASH acetyl-CoA + carnitine => acetylcarnitine + CoASH 4,8,12-trimethyltridecanoyl-CoA + 2 O2 + 2 H2O + 2 NAD+ + 2 CoASH => 4,8-dimethylnonanoyl-CoA + 2 H2O2 + 2 NADH + 2 H+ + acetyl-CoA + propionyl-CoA Beta-oxidation of pristanoyl-CoA Authored: D'Eustachio, P, 2009-03-16 18:50:14 Edited: D'Eustachio, P, 2009-03-18 13:33:40 GENE ONTOLOGYGO:0033540 Pristanoyl-CoA, generated in the peroxisome by alpha-oxidation of dietary phytanic acid, is further catabolized by three cycles of peroxisomal beta-oxidation to yield 4,8-dimethylnonanoyl-CoA, acetyl-CoA and two molecules of propionyl-CoA. These molecules in turn are converted to carnitine conjugates, which can be transported to mitochondria (Wanders and Waterham 2006, Verhoeven et al. 1998, Ferdinandusse et al. 1999). Pubmed10486279 Pubmed16756494 Pubmed9469587 Reactome Database ID Release 43389887 Reactome, http://www.reactome.org ReactomeREACT_17017 Reviewed: Jassal, B, 2009-02-27 15:21:18