phytanic acid


Summary: A 20-carbon branched chain fatty acid. In phytanic acid storage disease (REFSUM DISEASE) this lipid may comprise as much as 30% of the total fatty acids of the plasma. This is due to a phytanic acid alpha-hydroxylase deficiency.

Top Publications

  1. van den Brink D, Wanders R. Phytanic acid: production from phytol, its breakdown and role in human disease. Cell Mol Life Sci. 2006;63:1752-65 pubmed
    b>Phytanic acid is a branched-chain fatty acid that accumulates in a variety of metabolic disorders. High levels of phytanic acid found in patients can exceed the millimolar range and lead to severe symptoms...
  2. Allen N, Grace P, Ginn A, Travis R, Roddam A, Appleby P, et al. Phytanic acid: measurement of plasma concentrations by gas-liquid chromatography-mass spectrometry analysis and associations with diet and other plasma fatty acids. Br J Nutr. 2008;99:653-9 pubmed
    ..It is hypothesised that phytanic acid, a C20 branched-chain fatty acid found predominantly in foods from ruminant animals, may be involved in early ..
  3. McCarty M. Up-regulation of PPARgamma coactivator-1alpha as a strategy for preventing and reversing insulin resistance and obesity. Med Hypotheses. 2005;64:399-407 pubmed
    ..b>Phytanic acid, a branched-chain fatty acid found in omnivore diets, can also activate PPARdelta, and thus should be examined ..
  4. Barth P, Wanders R, Schutgens R, Staalman C. Variant rhizomelic chondrodysplasia punctata (RCDP) with normal plasma phytanic acid: clinico-biochemical delineation of a subtype and complementation studies. Am J Med Genet. 1996;62:164-8 pubmed
    ..chondrodysplasia calcificans punctata (RCDP) is an autosomal recessive peroxisomal disorder which affects phytanic acid oxidation and de novo biosynthesis of plasmalogens in liver and fibroblasts...
  5. Jansen G, Mihalik S, Watkins P, Moser H, Jakobs C, Denis S, et al. Phytanoyl-CoA hydroxylase is present in human liver, located in peroxisomes, and deficient in Zellweger syndrome: direct, unequivocal evidence for the new, revised pathway of phytanic acid alpha-oxidation in humans. Biochem Biophys Res Commun. 1996;229:205-10 pubmed
    b>Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) is a branched-chain fatty acid which accumulates in a number of inherited diseases in human...
  6. Vreken P, van Lint A, Bootsma A, Overmars H, Wanders R, Van Gennip A. Rapid stable isotope dilution analysis of very-long-chain fatty acids, pristanic acid and phytanic acid using gas chromatography-electron impact mass spectrometry. J Chromatogr B Biomed Sci Appl. 1998;713:281-7 pubmed
    ..of most peroxisomal disorders is the accumulation of very-long-chain fatty acids (VLCFAs) and/or pristanic and phytanic acid in plasma...
  7. Kahlert S, Schonfeld P, Reiser G. The Refsum disease marker phytanic acid, a branched chain fatty acid, affects Ca2+ homeostasis and mitochondria, and reduces cell viability in rat hippocampal astrocytes. Neurobiol Dis. 2005;18:110-8 pubmed
    The saturated branched chain fatty acid, phytanic acid, a degradation product of chlorophyll, accumulates in Refsum disease, an inherited peroxisomal disorder with neurological clinical features...
  8. Gootjes J, Mooijer P, Dekker C, Barth P, Poll The B, Waterham H, et al. Biochemical markers predicting survival in peroxisome biogenesis disorders. Neurology. 2002;59:1746-9 pubmed
    ..acyltransferase (DHAPAT) activity, C26:0/C22:0 ratio, C26:0 and pristanic acid beta-oxidation, and phytanic acid alpha-oxidation were analyzed in fibroblasts from a series of patients with defined clinical phenotypes...
  9. Jansen G, Ofman R, Denis S, Ferdinandusse S, Hogenhout E, Jakobs C, et al. Phytanoyl-CoA hydroxylase from rat liver. Protein purification and cDNA cloning with implications for the subcellular localization of phytanic acid alpha-oxidation. J Lipid Res. 1999;40:2244-54 pubmed
    ..PhyH) catalyzes the conversion of phytanoyl-CoA to 2-hydroxyphytanoyl-CoA, which is the first step in the phytanic acid alpha-oxidation pathway...

More Information


  1. Wierzbicki A, Lloyd M, Schofield C, Feher M, Gibberd F. Refsum's disease: a peroxisomal disorder affecting phytanic acid alpha-oxidation. J Neurochem. 2002;80:727-35 pubmed
    ..include retinitis pigmentosa, blindness, anosmia, deafness, sensory neuropathy, ataxia and accumulation of phytanic acid in plasma- and lipid-containing tissues...
  2. Werner L, Hellgren L, Raff M, Jensen S, Petersen R, Drachmann T, et al. Effect of dairy fat on plasma phytanic acid in healthy volunteers - a randomized controlled study. Lipids Health Dis. 2011;10:95 pubmed publisher
    b>Phytanic acid produced in ruminants from chlorophyll may have preventive effects on the metabolic syndrome, partly due to its reported RXR and PPAR- ? agonist activity...
  3. Elmazar M, Nau H. Potentiation of the teratogenic effects induced by coadministration of retinoic acid or phytanic acid/phytol with synthetic retinoid receptor ligands. Arch Toxicol. 2004;78:660-8 pubmed
    ..A non-teratogenic dose of the natural RXR agonist phytanic acid (100 mg/kg orally) or its precursor phytol (500 mg/kg orally) was coadministered with a synthetic RARalpha-..
  4. Atshaves B, McIntosh A, Landrock D, Payne H, Mackie J, Maeda N, et al. Effect of SCP-x gene ablation on branched-chain fatty acid metabolism. Am J Physiol Gastrointest Liver Physiol. 2007;292:G939-51 pubmed
    ..In summary, the present work with SCP-x gene-ablated mice demonstrates, for the first time, a direct physiological relationship between lack of SCP-x and decreased ability to metabolize branched-chain lipids. ..
  5. Little J, Williams L, Xu J, Radominska Pandya A. Glucuronidation of the dietary fatty acids, phytanic acid and docosahexaenoic acid, by human UDP-glucuronosyltransferases. Drug Metab Dispos. 2002;30:531-3 pubmed
    ..In the present study, the dietary fatty acids (FA), phytanic acid (PA), and docosahexaenoic acid (DHA) have been used as substrates for human UDP-glucuronosyltransferases (UGTs)..
  6. Schluter A, Barberà M, Iglesias R, Giralt M, Villarroya F. Phytanic acid, a novel activator of uncoupling protein-1 gene transcription and brown adipocyte differentiation. Biochem J. 2002;362:61-9 pubmed
    b>Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) is a phytol-derived branched-chain fatty acid present in dietary products...
  7. Wierzbicki A. Peroxisomal disorders affecting phytanic acid alpha-oxidation: a review. Biochem Soc Trans. 2007;35:881-6 pubmed
    ..of these pathways and the diseases associated with mutations in enzymes involved in the degradation of phytanic acid have led to the clarification of the pathophysiology of Refsum's disease, rhizomelic chondrodysplasia and ..
  8. Komen J, Distelmaier F, Koopman W, Wanders R, Smeitink J, Willems P. Phytanic acid impairs mitochondrial respiration through protonophoric action. Cell Mol Life Sci. 2007;64:3271-81 pubmed
    ..inherited neurodegenerative disorder characterized by accumulation of the dietary branched-chain fatty acid phytanic acid in plasma and tissues caused by a defect in the alphaoxidation pathway...
  9. Lampen A, Meyer S, Nau H. Phytanic acid and docosahexaenoic acid increase the metabolism of all-trans-retinoic acid and CYP26 gene expression in intestinal cells. Biochim Biophys Acta. 2001;1521:97-106 pubmed
    ..The effects of the food compounds phytol, the phytol metabolite phytanic acid, and the fatty acid docosahexaenoic acid (DHA) on the retinoid signaling pathway in intestinal cells were ..
  10. Al Dirbashi O, Santa T, Rashed M, Al Hassnan Z, Shimozawa N, Chedrawi A, et al. Rapid UPLC-MS/MS method for routine analysis of plasma pristanic, phytanic, and very long chain fatty acid markers of peroxisomal disorders. J Lipid Res. 2008;49:1855-62 pubmed publisher
    Quantification of pristanic acid, phytanic acid, and very long chain fatty acids (i.e., hexacosanoic, tetracosanoic, and docosanoic acids) in plasma is the primary method for investigateing a multitude of peroxisomal disorders (PDs)...
  11. Price A, Allen N, Appleby P, Crowe F, Jenab M, Rinaldi S, et al. Plasma phytanic acid concentration and risk of prostate cancer: results from the European Prospective Investigation into Cancer and Nutrition. Am J Clin Nutr. 2010;91:1769-76 pubmed publisher
    b>Phytanic acid, a fatty acid predominantly obtained from foods high in ruminant fat, may have a biological role in the up-regulation of the protein alpha-methylacyl-coenzyme A racemase, which is overexpressed in prostate cancer tissue...
  12. Verhoeven N, Jakobs C. Human metabolism of phytanic acid and pristanic acid. Prog Lipid Res. 2001;40:453-66 pubmed
    b>Phytanic acid is a methyl-branched fatty acid present in the human diet. Due to its structure, degradation by beta-oxidation is impossible. Instead, phytanic acid is oxidized by alpha-oxidation, yielding pristanic acid...
  13. Wanders R, Komen J. Peroxisomes, Refsum's disease and the alpha- and omega-oxidation of phytanic acid. Biochem Soc Trans. 2007;35:865-9 pubmed
    In the present paper, we describe the current state of knowledge regarding the enzymology of the phytanic acid alpha-oxidation pathway. The product of phytanic acid alpha-oxidation, i.e...
  14. McCarty M. The chlorophyll metabolite phytanic acid is a natural rexinoid--potential for treatment and prevention of diabetes. Med Hypotheses. 2001;56:217-9 pubmed
    ..The chlorophyll metabolite phytanic acid has been shown to be a natural ligand for RXR, active in concentrations near its physiological levels...
  15. Schluter A, Giralt M, Iglesias R, Villarroya F. Phytanic acid, but not pristanic acid, mediates the positive effects of phytol derivatives on brown adipocyte differentiation. FEBS Lett. 2002;517:83-6 pubmed
    The phytol derivatives phytanic acid and pristanic acid may activate nuclear hormone receptors and influence gene expression and cell differentiation. Phytanic acid induces brown adipocyte differentiation...
  16. Zomer A, van der Burg B, Jansen G, Wanders R, Poll The B, van der Saag P. Pristanic acid and phytanic acid: naturally occurring ligands for the nuclear receptor peroxisome proliferator-activated receptor alpha. J Lipid Res. 2000;41:1801-7 pubmed
    b>Phytanic acid and pristanic acid are branched-chain fatty acids, present at micromolar concentrations in the plasma of healthy individuals...
  17. Mönnig G, Wiekowski J, Kirchhof P, Stypmann J, Plenz G, Fabritz L, et al. Phytanic acid accumulation is associated with conduction delay and sudden cardiac death in sterol carrier protein-2/sterol carrier protein-x deficient mice. J Cardiovasc Electrophysiol. 2004;15:1310-6 pubmed
    ..Left ventricular function studied by echocardiography was similar in both strains. Phytanic acid but not pristanic acid accumulated in the phospholipid fraction of myocardial membranes isolated from SCP2 ..
  18. Wanders R, Jansen G, Lloyd M. Phytanic acid alpha-oxidation, new insights into an old problem: a review. Biochim Biophys Acta. 2003;1631:119-35 pubmed
    b>Phytanic acid (3,7,10,14-tetramethylhexadecanoic acid) is a branched-chain fatty acid which is known to accumulate in a number of different genetic diseases including Refsum disease...
  19. Schonfeld P, Reiser G. Rotenone-like action of the branched-chain phytanic acid induces oxidative stress in mitochondria. J Biol Chem. 2006;281:7136-42 pubmed
    b>Phytanic acid (Phyt) increase is associated with the hereditary neurodegenerative Refsum disease. To elucidate the still unclear toxicity of Phyt, mitochondria from brain and heart of adult rats were exposed to free Phyt...
  20. Goto T, Takahashi N, Kato S, Egawa K, Ebisu S, Moriyama T, et al. Phytol directly activates peroxisome proliferator-activated receptor alpha (PPARalpha) and regulates gene expression involved in lipid metabolism in PPARalpha-expressing HepG2 hepatocytes. Biochem Biophys Res Commun. 2005;337:440-5 pubmed
    ..Because PPARalpha activation enhances circulating lipid clearance, phytol may be important in managing abnormalities in lipid metabolism. ..
  21. Rönicke S, Kruska N, Kahlert S, Reiser G. The influence of the branched-chain fatty acids pristanic acid and Refsum disease-associated phytanic acid on mitochondrial functions and calcium regulation of hippocampal neurons, astrocytes, and oligodendrocytes. Neurobiol Dis. 2009;36:401-10 pubmed publisher
    Pristanic acid and phytanic acid are branched-chain fatty acids, which play an important role in diseases with peroxisomal impairment, like Refsum disease (MIM 266500), Zellwegers syndrome and alpha-methylacyl-CoA racemase deficiency (..
  22. Skjeldal O, Stokke O, Refsum S, Norseth J, Petit H. Clinical and biochemical heterogeneity in conditions with phytanic acid accumulation. J Neurol Sci. 1987;77:87-96 pubmed
    b>Phytanic acid accumulation has for more than 20 years been used as a diagnostic criterion of Refsum's disease...
  23. Leiber F, Kreuzer M, Nigg D, Wettstein H, Scheeder M. A study on the causes for the elevated n-3 fatty acids in cows' milk of alpine origin. Lipids. 2005;40:191-202 pubmed
    ..lowland periods whereas the trans-11, cis-13 isomer further increased with altitude. Long-chain n-3 FA and phytanic acid increased while arachidonic acid decreased with grass-only feeding, but none of them responded to altitude...
  24. Thornburg T, Turner A, Chen Y, Vitolins M, Chang B, Xu J. Phytanic acid, AMACR and prostate cancer risk. Future Oncol. 2006;2:213-23 pubmed
    ..A greater understanding of such relationships should provide better ways to establish overall risk, to screen for the disease and perhaps to offer specific opportunities for prevention and treatment. ..
  25. Tang X, Suh M, Li R, Gudas L. Cell proliferation inhibition and alterations in retinol esterification induced by phytanic acid and docosahexaenoic acid. J Lipid Res. 2007;48:165-76 pubmed
    We investigated the effects of two natural dietary retinoid X receptor (RXR) ligands, phytanic acid (PA) and docosahexaenoic acid (DHA), on proliferation and on the metabolism of retinol (vitamin A) in both cultured normal human prostate ..
  27. Ellinghaus P, Wolfrum C, Assmann G, Spener F, Seedorf U. Phytanic acid activates the peroxisome proliferator-activated receptor alpha (PPARalpha) in sterol carrier protein 2-/ sterol carrier protein x-deficient mice. J Biol Chem. 1999;274:2766-72 pubmed
    ..protein x-gene (Scp2) leads to defective peroxisomal catabolism of 3,7,11, 15-tetramethylhexadecanoic acid (phytanic acid), peroxisome proliferation, hypolipidemia, and enhanced hepatic expression of several genes that have been ..
  28. Wanders R, Jansen G, Skjeldal O. Refsum disease, peroxisomes and phytanic acid oxidation: a review. J Neuropathol Exp Neurol. 2001;60:1021-31 pubmed
    ..The discovery of markedly elevated levels of the branched-chain fatty acid phytanic acid in certain patients marked Refsum disease as a disorder of lipid metabolism...
  29. Mukherji M, Kershaw N, Schofield C, Wierzbicki A, Lloyd M. Utilization of sterol carrier protein-2 by phytanoyl-CoA 2-hydroxylase in the peroxisomal alpha oxidation of phytanic acid. Chem Biol. 2002;9:597-605 pubmed
    Since it possesses a 3-methyl group, phytanic acid is degraded by a peroxisomal alpha-oxidation pathway, the first step of which is catalyzed by phytanoyl-CoA 2-hydroxylase (PAHX)...
  30. Radominska Pandya A, Chen G. Photoaffinity labeling of human retinoid X receptor beta (RXRbeta) with 9-cis-retinoic acid: identification of phytanic acid, docosahexaenoic acid, and lithocholic acid as ligands for RXRbeta. Biochemistry. 2002;41:4883-90 pubmed
    ..However, the saturated diterpenoid phytanic acid (PA) and docosahexaenoic acid, which have been recently shown to activate the nuclear receptor, RXR, competed ..
  31. Xu J, Thornburg T, Turner A, Vitolins M, Case D, Shadle J, et al. Serum levels of phytanic acid are associated with prostate cancer risk. Prostate. 2005;63:209-14 pubmed
    ..between prostate cancer risk and over-consumption of dairy products and red meat, indirectly suggest that phytanic acid, which primarily comes from dietary intake of dairy and red meat and requires the AMACR enzyme for its ..
  32. Elmazar M, El Abhar H, Schaalan M, Farag N. Phytol/Phytanic acid and insulin resistance: potential role of phytanic acid proven by docking simulation and modulation of biochemical alterations. PLoS ONE. 2013;8:e45638 pubmed publisher
    ..with RXR, we aimed to test the potential antidiabetic effect of phytol (250 mg/kg), the natural precursor of phytanic acid, a RXR ligand and/or pioglitazone (5 mg/kg) to diabetic insulin-resistant rats...
  33. Ollberding N, Aschebrook Kilfoy B, Caces D, Wright M, Weisenburger D, Smith S, et al. Phytanic acid and the risk of non-Hodgkin lymphoma. Carcinogenesis. 2013;34:170-5 pubmed publisher
    ..b>Phytanic acid, a saturated fatty acid obtained primarily through the consumption of ruminant meat and dairy products, may ..
  34. Kruska N, Reiser G. Phytanic acid and pristanic acid, branched-chain fatty acids associated with Refsum disease and other inherited peroxisomal disorders, mediate intracellular Ca2+ signaling through activation of free fatty acid receptor GPR40. Neurobiol Dis. 2011;43:465-72 pubmed publisher
    The accumulation of the two branched-chain fatty acids phytanic acid and pristanic acid is known to play an important role in several diseases with peroxisomal impairment, like Refsum disease, Zellweger syndrome and ?-methylacyl-CoA ..
  35. Ferdinandusse S, Zomer A, Komen J, van den Brink C, Thanos M, Hamers F, et al. Ataxia with loss of Purkinje cells in a mouse model for Refsum disease. Proc Natl Acad Sci U S A. 2008;105:17712-7 pubmed publisher
    ..of the peroxisomal alpha-oxidation system, resulting in the accumulation of the branched-chain fatty acid phytanic acid. The main clinical symptoms are polyneuropathy, cerebellar ataxia, and retinitis pigmentosa...
  36. Schonfeld P, Reiser G. Comment concerning the article: 'Phytanic acid impairs mitochondrial respiration through protonophoric action' by Komen et al.: branched chain phytanic acid inhibits the activity of the mitochondrial respiratory chain. Cell Mol Life Sci. 2008;65:2266-9 pubmed publisher
  37. Jansen G, van den Brink D, Ofman R, Draghici O, Dacremont G, Wanders R. Identification of pristanal dehydrogenase activity in peroxisomes: conclusive evidence that the complete phytanic acid alpha-oxidation pathway is localized in peroxisomes. Biochem Biophys Res Commun. 2001;283:674-9 pubmed
    b>Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) is a branched-chain fatty acid which, due to the methyl-group at the 3-position, can not undergo beta-oxidation unless the terminal carboxyl-group is removed by alpha-oxidation...
  38. Takemoto Y, Suzuki Y, Horibe R, Shimozawa N, Wanders R, Kondo N. Gas chromatography/mass spectrometry analysis of very long chain fatty acids, docosahexaenoic acid, phytanic acid and plasmalogen for the screening of peroxisomal disorders. Brain Dev. 2003;25:481-7 pubmed
    Very long chain fatty acids (VLCFAs) and docosahexaenoic acid (DHA), phytanic acid, and plasmalogens are usually measured individually...
  39. Hashimoto T, Shimizu N, Kimura T, Takahashi Y, Ide T. Polyunsaturated fats attenuate the dietary phytol-induced increase in hepatic fatty acid oxidation in mice. J Nutr. 2006;136:882-6 pubmed
    ..The hepatic concentration of phytanic acid, a metabolite of phytol that is the ligand and activator of retinoid X receptors and peroxisome proliferator-..
  40. Heim M, Johnson J, Boess F, Bendik I, Weber P, Hunziker W, et al. Phytanic acid, a natural peroxisome proliferator-activated receptor (PPAR) agonist, regulates glucose metabolism in rat primary hepatocytes. FASEB J. 2002;16:718-20 pubmed
    b>Phytanic acid, a metabolite of the chlorophyll molecule, is part of the human diet and is present in normal human serum at low micromolar concentrations...
  41. Wright M, Bowen P, Virtamo J, Albanes D, Gann P. Estimated phytanic acid intake and prostate cancer risk: a prospective cohort study. Int J Cancer. 2012;131:1396-406 pubmed publisher
    b>Phytanic acid is a saturated fatty acid found predominantly in red meat and dairy products and may contribute to increases in prostate cancer risk that are observed with higher intakes of these foods...
  42. Wanders R, Komen J, Ferdinandusse S. Phytanic acid metabolism in health and disease. Biochim Biophys Acta. 2011;1811:498-507 pubmed publisher
    b>Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) is a branched-chain fatty acid which cannot be beta-oxidized due to the presence of the first methyl group at the 3-position...
  43. van den Brink D, van Miert J, Dacremont G, Rontani J, Jansen G, Wanders R. Identification of fatty aldehyde dehydrogenase in the breakdown of phytol to phytanic acid. Mol Genet Metab. 2004;82:33-7 pubmed
    ..In its free form, phytol is metabolized to phytanic acid, which accumulates in patients suffering from a variety of peroxisomal disorders, including Refsum disease...
  44. Watkins P, Moser A, Toomer C, Steinberg S, Moser H, Karaman M, et al. Identification of differences in human and great ape phytanic acid metabolism that could influence gene expression profiles and physiological functions. BMC Physiol. 2010;10:19 pubmed publisher
    ..To investigate functional genomic consequences of these differences, we compared their physiological levels of phytanic acid, a branched chain fatty acid that can be derived from the microbial degradation of chlorophyll in ruminant ..
  45. Hellgren L. Phytanic acid--an overlooked bioactive fatty acid in dairy fat?. Ann N Y Acad Sci. 2010;1190:42-9 pubmed publisher
    b>Phytanic acid is a multibranched fatty acid with reported retinoid X receptor (RXR) and peroxisome proliferator-activated receptor-alpha (PPAR-alpha) agonist activity, which have been suggested to have preventive effects on metabolic ..
  46. Wolfrum C, Ellinghaus P, Fobker M, Seedorf U, Assmann G, Borchers T, et al. Phytanic acid is ligand and transcriptional activator of murine liver fatty acid binding protein. J Lipid Res. 1999;40:708-14 pubmed
    Branched-chain phytanic acid is metabolized in liver peroxisomes...
  47. Schluter A, Yubero P, Iglesias R, Giralt M, Villarroya F. The chlorophyll-derived metabolite phytanic acid induces white adipocyte differentiation. Int J Obes Relat Metab Disord. 2002;26:1277-80 pubmed
    b>Phytanic acid is a derivative of the phytol side-chain of chlorophyll. It appears in humans following the ingestion of fat-containing foods and is present in human blood at a low micromolar concentration...
  48. Powers J, Kenjarski T, Moser A, Moser H. Cerebellar atrophy in chronic rhizomelic chondrodysplasia punctata: a potential role for phytanic acid and calcium in the death of its Purkinje cells. Acta Neuropathol. 1999;98:129-34 pubmed
    ..Elevated serum/CSF phytanic acid, decreased levels of tissue plasmalogens and increased chronological age are believed to play synergistic ..
  49. Sathe M, Derveni M, Allen M, Cullen D. Use of polystyrene-supported 2-isobutoxy-1-isobutoxycarbonyl-1,2-dihydroquinoline for the preparation of a hapten-protein conjugate for antibody development. Bioorg Med Chem Lett. 2010;20:1792-5 pubmed publisher
    ..coupling reagent, was successfully employed for the first time in the bioconjugation of an example hapten (phytanic acid derivative) to a carrier protein (bovine serum albumin (BSA)) within the context of immunogen preparation for ..
  50. Westin M, Hunt M, Alexson S. Peroxisomes contain a specific phytanoyl-CoA/pristanoyl-CoA thioesterase acting as a novel auxiliary enzyme in alpha- and beta-oxidation of methyl-branched fatty acids in mouse. J Biol Chem. 2007;282:26707-16 pubmed
    b>Phytanic acid and pristanic acid are derived from phytol, which enter the body via the diet...
  51. Fukasawa T, Murashima K, Nemoto T, Matsumoto I, Koga J, Kubota H, et al. Identification of marker genes for lipid-lowering effect of a short-chain fructooligosaccharide by DNA microarray analysis. J Diet Suppl. 2009;6:254-62 pubmed publisher
    ..05). Since Lpl is involved in lipid metabolism, the up-regulation of Lpl in the liver can be a potential marker of the lipid-lowering effect of scFOS. ..
  52. Jansen G, Wanders R. Alpha-oxidation. Biochim Biophys Acta. 2006;1763:1403-12 pubmed
    b>Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) is a branched chain fatty acid, which is a constituent of the human diet. The presence of the 3-methyl group of phytanic acid prevents degradation by beta-oxidation...
  53. Singh I. Peroxisomal fatty acid oxidation and cellular redox. Methods Enzymol. 2002;352:361-72 pubmed
  54. Young J, Therkildsen M, Ekstrand B, Che B, Larsen M, Oksbjerg N, et al. Novel aspects of health promoting compounds in meat. Meat Sci. 2013;95:904-11 pubmed publisher
    ..In addition, meat could be an important source of phytanic acid, conjugated linoleic acids and antioxidants...
  55. McMillan H, Worthylake T, Schwartzentruber J, Gottlieb C, Lawrence S, MacKenzie A, et al. Specific combination of compound heterozygous mutations in 17?-hydroxysteroid dehydrogenase type 4 (HSD17B4) defines a new subtype of D-bifunctional protein deficiency. Orphanet J Rare Dis. 2012;7:90 pubmed publisher
    ..Biochemical analysis revealed normal levels of plasma VLCFA, phytanic acid and pristanic acid, and normal bile acids in urine; based on these results no diagnosis was made...
  56. Lloyd M, Mukherji M, Kershaw N, Chien W, Wierzbicki A, Schofield C. Role of phytanoyl-CoA 2-hydroxylase in phytanic acid metabolism. Adv Exp Med Biol. 2003;544:303-4 pubmed
  57. Singh H, Brogan M, Johnson D, Poulos A. Peroxisomal beta-oxidation of branched chain fatty acids in human skin fibroblasts. J Lipid Res. 1992;33:1597-605 pubmed
  58. Baldwin E, Gibberd F, Harley C, Sidey M, Feher M, Wierzbicki A. The effectiveness of long-term dietary therapy in the treatment of adult Refsum disease. J Neurol Neurosurg Psychiatry. 2010;81:954-7 pubmed publisher
    ..To evaluate the long-term effectiveness of dietary therapy with regular dietetic reinforcement for adult Refsum disease...