Page last updated: 2024-09-03

pristanic acid and phytanic acid

pristanic acid has been researched along with phytanic acid in 64 studies

Compound Research Comparison

Studies
(pristanic acid)
Trials
(pristanic acid)
Recent Studies (post-2010)
(pristanic acid)
Studies
(phytanic acid)
Trials
(phytanic acid)
Recent Studies (post-2010) (phytanic acid)
103217435370

Research

Studies (64)

TimeframeStudies, this research(%)All Research%
pre-19903 (4.69)18.7374
1990's32 (50.00)18.2507
2000's18 (28.13)29.6817
2010's10 (15.63)24.3611
2020's1 (1.56)2.80

Authors

AuthorsStudies
Hancock, AJ; Kates, M1
Jakobs, C; Kneer, J; Kok, RM; Poll-The, BT; Saudubray, JM; Schor, DS; Stellaard, F; ten Brink, HJ1
Jakobs, C; Kok, RM; Schor, DS; Stellaard, F; ten Brink, HJ; van den Heuvel, CM; Wanders, RJ1
Chow, CW; Christodoulou, J; Danks, DM; Fellenberg, AJ; Poulos, A1
Jakobs, C; Kok, RM; Poll-The, BT; Schor, DS; ten Brink, HJ; Wanders, RJ1
Kalipada, P; Lazo, O; Singh, AK; Singh, I1
Brogan, M; Johnson, D; Poulos, A; Singh, H1
Jakobs, C; Schutgens, RB; Stellaard, F; ten Brink, HJ; Wanders, RJ1
Jakobs, C; Poll-The, BT; Saudubray, JM; ten Brink, HJ; Wanders, RJ1
Kase, BF; Olund, J; Sisfontes, L1
Fellenberg, AJ; Johnson, DW; Poulos, A; Sharp, P1
Applegarth, DA; Clarke, LA; Harris, HM; Wong, J1
Barbosa, E; Pahan, K; Singh, AK; Singh, I1
Molzer, B; Schmitt, K; Stöckler, S; Tulzer, G; Tulzer, W1
Eyssen, HJ; Huang, S; Mannaerts, GP; Van Veldhoven, PP1
Gulati, S; Pahan, K; Singh, I1
Poulos, A; Singh, H1
Brandt, NJ; Christensen, E; Jakobs, C; ten Brink, HJ; Wanders, RJ1
Beckman, K; Poulos, A; Singh, H1
Christensen, E; Jakobs, C; Largillière, C; ten Brink, HJ; van den Heuvel, CM1
Crane, DI; Paton, BC; Poulos, A; Sharp, PC1
Björkhem, I; Kase, BF1
Casteels, M; Clayton, PT; Johnson, AW; Lynes, GW; Mannaerts, G; Mills, KA; Wilson, J1
Lima, MR; Mota, CR; Nogueira, A; Vilarinho, A1
Jakobs, C; Kulik, W; van den Heuvel, CM; Verhoeven, NM1
Cocquyt, G; Dacremont, G; Vincent, G1
Jakobs, C; Schor, DS; ten Brink, HJ; Verhoeven, NM; Wanders, RJ1
Christensen, E; Jakobs, C; Leth, H; Pedersen, SA; Schutgens, RB; Wanders, RJ1
Jakobs, C; Jansen, GA; Schor, DS; Verhoeven, NM; Wanders, RJ1
Jakobs, C; Kok, RM; Roe, CR; Roe, DS; Verhoeven, NM; Wanders, RJ1
Carney, G; Jakobs, C; Rizzo, WB; Somers, MP; Verhoeven, NM; Wanders, RJ1
Bootsma, AH; Overmars, H; van Gennip, AH; van Lint, AE; Vreken, P; Wanders, RJ1
Jakobs, C; Poll-The, BT; Saudubray, JM; Verhoeven, NM; Wanders, RJ1
Jakobs, C; Roe, CR; ten Brink, HJ; Verhoeven, NM; Wanders, RJ1
Kim, DG; Mihalik, SJ; Steinberg, SJ; Wang, SJ; Watkins, PA1
Jansen, GA; Poll-The, BT; van Der Burg, B; van Der Saag, PT; Wanders, RJ; Zomer, AW1
Jakobs, C; Verhoeven, NM1
Dacremont, G; Ferdinandusse, S; Rusch, H; van Lint, AE; Vreken, P; Wanders, RJ1
Giralt, M; Iglesias, R; Schluter, A; Villarroya, F1
Barth, PG; Dekker, C; Gootjes, J; Mooijer, PA; Poll-The, BT; Wanders, RJ; Waterham, HR2
Gäckler, D; Kingreen, H; Klingel, R; Muselmann, L; Straube, R; Thiele, A1
Evans, J; Ho, SM; Jiang, Z; L'Esperance, BS; Lam, YW; Leav, I; Mobley, JA; Wotkowitz, C; Zielie, P1
Johnson, DW; Oe, T; Trinh, MU1
Poll-The, BT; van der Saag, PT; Zomer, AW1
Dacremont, G; Jansen, GA; Rontani, JF; van den Brink, DM; van Miert, JN; Wanders, RJ1
Baumgartner, MR; Bonetti, G; Jakobs, C; Nassogne, MC; Peduto, A; Poll-The, BT; Rabier, D; Saudubray, JM; Spada, M; Verhoeven, NM1
Alger, S; Burdon, MA; Chavda, S; Clarke, CE; Denis, S; Ferdinandusse, S; Preece, MA; Wanders, RJ1
Atshaves, BP; Ball, J; Kier, AB; Landrock, D; Mackie, JT; Maeda, N; McIntosh, AL; Payne, HR; Schroeder, F1
Alexson, SEH; Hunt, MC; Westin, MAK1
Al-Dirbashi, OY; Al-Hassnan, Z; Al-Mokhadab, M; Chedrawi, A; Jacob, M; Rashed, MS; Santa, T; Shimozawa, N1
Atshaves, BP; Kier, AB; Mackie, JT; McIntosh, AL; Payne, HR; Schroeder, F1
Kahlert, S; Kruska, N; Reiser, G; Rönicke, S1
Van Veldhoven, PP1
Freeman, WD; Gavrilov, DK; Matern, D; Oglesbee, D; Smith, EH; Tortorelli, S; Vavra, MW1
Kruska, N; Reiser, G1
Mamedov, IS; Novikov, PV; Smolina, IuA; Sukhorukov, VS1
Beaulieu, CL; Boycott, KM; Bulman, DE; Ferdinandusse, S; Geraghty, MT; Gottlieb, CC; Lawrence, SE; Mackenzie, A; Majewski, J; McMillan, HJ; Mooyer, PA; Schwartzentruber, J; Wanders, RJ; Worthylake, T1
Albanes, D; Gann, PH; Männistö, S; Moser, AB; Snyder, K; Weinstein, SJ; Wright, ME1
Briand, G; Cheillan, D; Feillet, F; Montaut-Verient, B; Schmitt, E; Sorlin, A; Wiedemann, A1
Antonetti, G; Bertini, E; Boenzi, S; Cappa, M; Dionisi-Vici, C; Rizzo, C; Semeraro, M1
Ferdinandusse, S; Herzog, K; van Lenthe, H; Vaz, FM; Wanders, RJA; Waterham, HR1
Asselberghs, S; Baes, M; De Schryver, E; Meyhi, E; Mezzar, S; Morvay, PL; Van Veldhoven, PP1
Bobe, G; Garzotto, M; Kopp, R; Shannon, J; Takata, Y; Zhang, Z1

Reviews

6 review(s) available for pristanic acid and phytanic acid

ArticleYear
The metabolism of phytanic acid and pristanic acid in man: a review.
    Journal of inherited metabolic disease, 1998, Volume: 21, Issue:7

    Topics: Animals; Fatty Acids; Humans; Oxidation-Reduction; Phytanic Acid

1998
Human metabolism of phytanic acid and pristanic acid.
    Progress in lipid research, 2001, Volume: 40, Issue:6

    Topics: Fatty Acids; Humans; Oxidation-Reduction; Peroxisomal Disorders; Peroxisomes; Phytanic Acid

2001
Phytanic and pristanic acid are naturally occuring ligands.
    Advances in experimental medicine and biology, 2003, Volume: 544

    Topics: Animals; Cell Line; Enzyme Induction; Fatty Acids; Humans; Ligands; Mixed Function Oxygenases; Peroxisomes; Phytanic Acid; Receptors, Cytoplasmic and Nuclear; Receptors, Retinoic Acid; Retinoid X Receptors; Transcription Factors

2003
Tremor and deep white matter changes in alpha-methylacyl-CoA racemase deficiency.
    Neurology, 2004, Jul-13, Volume: 63, Issue:1

    Topics: Brain; Combined Modality Therapy; Depressive Disorder; Dysarthria; Electroencephalography; Fatty Acids; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Paresis; Peroxisomal Disorders; Phytanic Acid; Plasma Exchange; Racemases and Epimerases; Reflex, Abnormal; Retinitis Pigmentosa; Treatment Failure; Tremor

2004
Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism.
    Journal of lipid research, 2010, Volume: 51, Issue:10

    Topics: Animals; Fatty Acids; Fatty Acids, Unsaturated; Humans; Lipid Metabolism; Oxidation-Reduction; Peroxisomal Disorders; Peroxisomes; Phytanic Acid

2010
Phytol and its metabolites phytanic and pristanic acids for risk of cancer: current evidence and future directions.
    European journal of cancer prevention : the official journal of the European Cancer Prevention Organisation (ECP), 2020, Volume: 29, Issue:2

    Topics: Animals; Butter; Carcinogenesis; Diet Surveys; Diet, Western; Dietary Supplements; Disease Models, Animal; Fatty Acids; Feeding Behavior; Humans; Neoplasms; Nuts; Phytanic Acid; Phytol; PPAR alpha; PPAR gamma; Retinoid X Receptors; Risk Assessment

2020

Trials

1 trial(s) available for pristanic acid and phytanic acid

ArticleYear
Rapid stable isotope dilution analysis of very-long-chain fatty acids, pristanic acid and phytanic acid using gas chromatography-electron impact mass spectrometry.
    Journal of chromatography. B, Biomedical sciences and applications, 1998, Aug-25, Volume: 713, Issue:2

    Topics: Deuterium; Fatty Acids; Female; Gas Chromatography-Mass Spectrometry; Humans; Indicator Dilution Techniques; Male; Peroxisomal Disorders; Phytanic Acid; Reproducibility of Results; Sensitivity and Specificity

1998

Other Studies

57 other study(ies) available for pristanic acid and phytanic acid

ArticleYear
Application of open-tubular gas-liquid chromatography in an investigation of the stability of pristanic and phytanic acids towards alkaline treatment of their methyl or [-] menthyl esters.
    Journal of chromatographic science, 1977, May-10, Volume: 15, Issue:5

    Topics: Alkalies; Chromatography, Gas; Drug Stability; Eicosanoic Acids; Fatty Acids; Hydrolysis; Phytanic Acid; Stereoisomerism

1977
In vivo study of phytanic acid alpha-oxidation in classic Refsum's disease and chondrodysplasia punctata.
    Pediatric research, 1992, Volume: 32, Issue:5

    Topics: Administration, Oral; Adult; Carbon Dioxide; Chondrodysplasia Punctata; Fatty Acids; Humans; Infant; Male; Oxidation-Reduction; Phytanic Acid; Refsum Disease

1992
Pristanic acid and phytanic acid in plasma from patients with peroxisomal disorders: stable isotope dilution analysis with electron capture negative ion mass fragmentography.
    Journal of lipid research, 1992, Volume: 33, Issue:1

    Topics: Aging; Child, Preschool; Fatty Acids; Gas Chromatography-Mass Spectrometry; Humans; Indicator Dilution Techniques; Infant; Infant, Newborn; Metabolism, Inborn Errors; Microbodies; Phytanic Acid; Refsum Disease

1992
Autopsy findings in two siblings with infantile Refsum disease.
    Acta neuropathologica, 1992, Volume: 83, Issue:2

    Topics: Adrenal Glands; Fatty Acids; Female; Humans; Infant; Liver; Microbodies; Phytanic Acid; Refsum Disease

1992
Phytanic acid alpha-oxidation: accumulation of 2-hydroxyphytanic acid and absence of 2-oxophytanic acid in plasma from patients with peroxisomal disorders.
    Journal of lipid research, 1992, Volume: 33, Issue:10

    Topics: Adolescent; Adult; Carbon Radioisotopes; Child; Child, Preschool; Fatty Acids; Gas Chromatography-Mass Spectrometry; Humans; Infant; Infant, Newborn; Middle Aged; Phytanic Acid; Refsum Disease

1992
Phytanic acid alpha-oxidation in human cultured skin fibroblasts.
    Biochimica et biophysica acta, 1992, Dec-10, Volume: 1180, Issue:2

    Topics: Cell Fractionation; Cells, Cultured; Coenzyme A Ligases; Epoxy Compounds; Fatty Acids; Fibroblasts; Humans; Oxidation-Reduction; Phytanic Acid; Repressor Proteins; Saccharomyces cerevisiae Proteins; Skin; Subcellular Fractions

1992
Peroxisomal beta-oxidation of branched chain fatty acids in human skin fibroblasts.
    Journal of lipid research, 1992, Volume: 33, Issue:11

    Topics: Antimycin A; Cells, Cultured; Digitonin; Electron Transport; Fatty Acids; Fibroblasts; Humans; Microbodies; Oxidation-Reduction; Phytanic Acid; Potassium Cyanide; Rotenone; Skin

1992
Pristanic acid and phytanic acid in plasma from patients with a single peroxisomal enzyme deficiency.
    Journal of inherited metabolic disease, 1991, Volume: 14, Issue:3

    Topics: Fatty Acids; Humans; Microbodies; Mixed Function Oxygenases; Oxidation-Reduction; Oxidoreductases; Phytanic Acid; Proteins; Refsum Disease

1991
Pristanic acid does not accumulate in peroxisomal acyl-CoA oxidase deficiency: evidence for a distinct peroxisomal pristanyl-CoA oxidase.
    Journal of inherited metabolic disease, 1991, Volume: 14, Issue:5

    Topics: Acyl-CoA Oxidase; Fatty Acids; Humans; Microbodies; Oxidation-Reduction; Oxidoreductases; Phytanic Acid

1991
Separation of phytanic and pristanic acid by high-pressure liquid chromatography: application of the method.
    Analytical biochemistry, 1991, Volume: 196, Issue:1

    Topics: Cells, Cultured; Chromatography, High Pressure Liquid; Fatty Acids; Humans; Phytanic Acid; Skin

1991
Accumulation of pristanic acid (2, 6, 10, 14 tetramethylpentadecanoic acid) in the plasma of patients with generalised peroxisomal dysfunction.
    European journal of pediatrics, 1988, Volume: 147, Issue:2

    Topics: Adrenoleukodystrophy; Chondrodysplasia Punctata; Fatty Acids; Humans; Lipidoses; Microbodies; Phytanic Acid; Refsum Disease

1988
Phytanic acid, pristanic acid, and very-long-chain fatty acid methyl esters measured simultaneously by capillary gas chromatography.
    Clinical chemistry, 1989, Volume: 35, Issue:4

    Topics: Chromatography, Gas; Eicosanoic Acids; Fatty Acids; Humans; Microbodies; Phytanic Acid

1989
Refsum disease: a defect in the alpha-oxidation of phytanic acid in peroxisomes.
    Journal of lipid research, 1993, Volume: 34, Issue:10

    Topics: Cells, Cultured; Coenzyme A Ligases; Endoplasmic Reticulum; Fatty Acids; Fibroblasts; Humans; Microbodies; Mitochondria; Oxidation-Reduction; Phytanic Acid; Refsum Disease

1993
[Zellweger syndrome, neonatal adrenoleukodystrophy or infantile Refsum's disease in a case with generalized peroxisome defect?].
    Wiener klinische Wochenschrift, 1993, Volume: 105, Issue:11

    Topics: Adrenoleukodystrophy; Catalase; Fatty Acids; Humans; Infant; Male; Microbodies; Phytanic Acid; Plasmalogens; Refsum Disease; Zellweger Syndrome

1993
The deficient degradation of synthetic 2- and 3-methyl-branched fatty acids in fibroblasts from patients with peroxisomal disorders.
    Journal of inherited metabolic disease, 1993, Volume: 16, Issue:2

    Topics: Adrenoleukodystrophy; Cell Line; Fatty Acids; Fibroblasts; Humans; Kinetics; Microbodies; Oxidation-Reduction; Palmitates; Phytanic Acid; Refsum Disease; X Chromosome; Zellweger Syndrome

1993
Phytanic acid alpha-oxidation in rat liver mitochondria.
    Biochimica et biophysica acta, 1994, Dec-15, Volume: 1201, Issue:3

    Topics: Animals; Carnitine; Clofibric Acid; Coenzyme A; Cytochrome P-450 Enzyme Inhibitors; Fatty Acids; Fibric Acids; Male; Mitochondria, Liver; Oxidation-Reduction; Phytanic Acid; Rats; Rats, Sprague-Dawley

1994
Substrate specificity of rat liver mitochondrial carnitine palmitoyl transferase I: evidence against alpha-oxidation of phytanic acid in rat liver mitochondria.
    FEBS letters, 1995, Feb-13, Volume: 359, Issue:2-3

    Topics: Animals; Biological Transport; Carnitine O-Palmitoyltransferase; Coenzyme A; Coenzyme A Ligases; Fatty Acids; In Vitro Techniques; Intracellular Membranes; Mitochondria, Liver; Oxidation-Reduction; Phytanic Acid; Rats; Repressor Proteins; Saccharomyces cerevisiae Proteins; Substrate Specificity

1995
Heterogeneity in di/trihydroxycholestanoic acidaemia.
    Annals of clinical biochemistry, 1994, Volume: 31 ( Pt 2)

    Topics: Cholestanols; Fatty Acids; Humans; Oxidoreductases; Phytanic Acid

1994
Peroxisomal beta-oxidation of branched chain fatty acids in rat liver. Evidence that carnitine palmitoyltransferase I prevents transport of branched chain fatty acids into mitochondria.
    The Journal of biological chemistry, 1994, Apr-01, Volume: 269, Issue:13

    Topics: Acyl Coenzyme A; Animals; Carnitine O-Palmitoyltransferase; Cells, Cultured; Fatty Acids; Fatty Acids, Nonesterified; Fibroblasts; Humans; Liver; Microbodies; Microsomes, Liver; Mitochondria, Liver; Oxidation-Reduction; Phytanic Acid; Rats; Skin

1994
Diagnosis of peroxisomal disorders by analysis of phytanic and pristanic acids in stored blood spots collected at neonatal screening.
    Clinical chemistry, 1993, Volume: 39, Issue:9

    Topics: Fatty Acids; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Microbodies; Neonatal Screening; Phytanic Acid

1993
Oxidation of pristanic acid in fibroblasts and its application to the diagnosis of peroxisomal beta-oxidation defects.
    The Journal of clinical investigation, 1996, Feb-01, Volume: 97, Issue:3

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Australia; Bile Acids and Salts; Cells, Cultured; Enoyl-CoA Hydratase; Fatty Acids; Fibroblasts; Genetic Complementation Test; Humans; Hybrid Cells; Isomerases; Microbodies; Multienzyme Complexes; Oxidation-Reduction; Peroxisomal Bifunctional Enzyme; Peroxisomal Disorders; Phytanic Acid; Prenatal Diagnosis; Zellweger Syndrome

1996
Studies on the degradation of [U-3H]-phytanic acid and [U-3H]-pristanic acid in cultured fibroblasts from children with peroxisomal disorders.
    Scandinavian journal of clinical and laboratory investigation, 1996, Volume: 56, Issue:3

    Topics: Cells, Cultured; Child; Fatty Acids; Fibroblasts; Humans; Microbodies; Phytanic Acid; Tritium; Zellweger Syndrome

1996
Ataxia associated with increased plasma concentrations of pristanic acid, phytanic acid and C27 bile acids but normal fibroblast branched-chain fatty acid oxidation.
    Journal of inherited metabolic disease, 1996, Volume: 19, Issue:6

    Topics: Ataxia; Bile Acids and Salts; Child; Fatty Acids; Female; Fibroblasts; Humans; Oxidation-Reduction; Phytanic Acid

1996
Rhizomelic chondrodysplasia punctata-like phenotype in a newborn male with normal peroxisomal function.
    Journal of pediatric orthopedics. Part B, 1997, Volume: 6, Issue:1

    Topics: Acyltransferases; Chondrodysplasia Punctata, Rhizomelic; Dihydroxyacetone Phosphate; Dwarfism; Fatal Outcome; Fatty Acids; Humans; Infant, Newborn; Male; Phenotype; Phytanic Acid; Plasmalogens; Radiography; Respiratory Insufficiency

1997
Pre- and postnatal diagnosis of peroxisomal disorders using stable-isotope dilution gas chromatography--mass spectrometry.
    Journal of inherited metabolic disease, 1995, Volume: 18 Suppl 1

    Topics: Amniotic Fluid; Bile Acids and Salts; Fatty Acids; Female; Gas Chromatography-Mass Spectrometry; Humans; Infant, Newborn; Peroxisomal Disorders; Phytanic Acid; Pipecolic Acids; Pregnancy; Prenatal Diagnosis; Radioisotope Dilution Technique; Reference Values

1995
Measurement of very long-chain fatty acids, phytanic and pristanic acid in plasma and cultured fibroblasts by gas chromatography.
    Journal of inherited metabolic disease, 1995, Volume: 18 Suppl 1

    Topics: Adrenoleukodystrophy; Cells, Cultured; Chromatography, Gas; Esters; Fatty Acids; Fibroblasts; Humans; Indicators and Reagents; Phytanic Acid; Solutions; Solvents

1995
Resolution of the phytanic acid alpha-oxidation pathway: identification of pristanal as product of the decarboxylation of 2-hydroxyphytanoyl-CoA.
    Biochemical and biophysical research communications, 1997, Aug-08, Volume: 237, Issue:1

    Topics: Aldehydes; Coenzyme A; Decarboxylation; Fatty Acids; Humans; Liver; Microbodies; Models, Chemical; Oxidation-Reduction; Phytanic Acid

1997
A new peroxisomal beta-oxidation disorder in twin neonates: defective oxidation of both cerotic and pristanic acids.
    Journal of inherited metabolic disease, 1997, Volume: 20, Issue:5

    Topics: Diseases in Twins; Fatty Acids; Humans; Infant, Newborn; Male; Microbodies; Oxidation-Reduction; Phytanic Acid; Zellweger Syndrome

1997
Phytanic acid alpha-oxidation: decarboxylation of 2-hydroxyphytanoyl-CoA to pristanic acid in human liver.
    Journal of lipid research, 1997, Volume: 38, Issue:10

    Topics: Biomarkers; Cell Fractionation; Coenzyme A; Decarboxylation; Fatty Acids; Humans; Liver; Microbodies; Microsomes; NAD; Phytanic Acid; Zellweger Syndrome

1997
Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts.
    Journal of lipid research, 1998, Volume: 39, Issue:1

    Topics: Carnitine Acyltransferases; Carnitine O-Palmitoyltransferase; Cells, Cultured; Fatty Acids; Fibroblasts; Gas Chromatography-Mass Spectrometry; Humans; Mass Spectrometry; Microbodies; Mitochondria; Oxidation-Reduction; Phytanic Acid; Zellweger Syndrome

1998
Involvement of microsomal fatty aldehyde dehydrogenase in the alpha-oxidation of phytanic acid.
    FEBS letters, 1998, Jun-16, Volume: 429, Issue:3

    Topics: Aldehyde Oxidoreductases; Aldehydes; Animals; CHO Cells; Cricetinae; Fatty Acids; Fibroblasts; Humans; Microsomes; Oxidation-Reduction; Phosphatidylethanolamines; Phytanic Acid; Recombinant Proteins; Sjogren-Larsson Syndrome

1998
Studies on the oxidation of phytanic acid and pristanic acid in human fibroblasts by acylcarnitine analysis.
    Journal of inherited metabolic disease, 1998, Volume: 21, Issue:7

    Topics: Carnitine; Cells, Cultured; Fatty Acids; Fibroblasts; Humans; Oxidation-Reduction; Peroxisomal Disorders; Phytanic Acid

1998
Human very-long-chain acyl-CoA synthetase: cloning, topography, and relevance to branched-chain fatty acid metabolism.
    Biochemical and biophysical research communications, 1999, Apr-13, Volume: 257, Issue:2

    Topics: Amino Acid Sequence; Animals; Base Sequence; Biomarkers; Cell Line; Cloning, Molecular; Coenzyme A Ligases; Endoplasmic Reticulum; Fatty Acids; Fluorescent Antibody Technique, Indirect; Humans; Liver; Microbodies; Molecular Sequence Data; Organ Specificity; Phytanic Acid; Rats; Sequence Homology, Amino Acid; Substrate Specificity; Transfection

1999
Pristanic acid and phytanic acid: naturally occurring ligands for the nuclear receptor peroxisome proliferator-activated receptor alpha.
    Journal of lipid research, 2000, Volume: 41, Issue:11

    Topics: Animals; Binding Sites; Cell Line; COS Cells; DNA; Drug Synergism; Fatty Acids; Fibroblasts; Haplorhini; Humans; Ligands; Phytanic Acid; Receptors, Cytoplasmic and Nuclear; Receptors, Retinoic Acid; Recombinant Fusion Proteins; Retinoid X Receptors; Transcription Factors; Transcriptional Activation

2000
Stereochemistry of the peroxisomal branched-chain fatty acid alpha- and beta-oxidation systems in patients suffering from different peroxisomal disorders.
    Journal of lipid research, 2002, Volume: 43, Issue:3

    Topics: Fatty Acids; Humans; Mixed Function Oxygenases; Oxidation-Reduction; Oxidoreductases; Peroxisomal Disorders; Phytanic Acid; Racemases and Epimerases; Refsum Disease; Stereoisomerism

2002
Phytanic acid, but not pristanic acid, mediates the positive effects of phytol derivatives on brown adipocyte differentiation.
    FEBS letters, 2002, Apr-24, Volume: 517, Issue:1-3

    Topics: Adipocytes; Adipose Tissue, Brown; Animals; Carrier Proteins; Cell Differentiation; Cold Temperature; Fatty Acids; Ion Channels; Membrane Proteins; Mice; Mitochondrial Proteins; Mixed Function Oxygenases; Phytanic Acid; Phytol; Promoter Regions, Genetic; RNA, Messenger; Thermogenesis; Uncoupling Protein 1

2002
Biochemical markers predicting survival in peroxisome biogenesis disorders.
    Neurology, 2002, Dec-10, Volume: 59, Issue:11

    Topics: Acyltransferases; Biomarkers; Fatty Acids; Fibroblasts; Humans; Oxidation-Reduction; Peroxisomal Disorders; Peroxisomes; Phenotype; Phytanic Acid; Plasmalogens; Predictive Value of Tests; Prognosis; Retrospective Studies; Survival; Zellweger Syndrome

2002
Membrane differential filtration is safe and effective for the long-term treatment of Refsum syndrome--an update of treatment modalities and pathophysiological cognition.
    Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis, 2003, Volume: 29, Issue:1

    Topics: Blood Component Removal; Diseases in Twins; Fatty Acids; Female; Filtration; Humans; Lipid Metabolism; Lipoproteins, LDL; Middle Aged; Mutation; Phytanic Acid; Picolinic Acids; Refsum Disease

2003
Branched fatty acids in dairy and beef products markedly enhance alpha-methylacyl-CoA racemase expression in prostate cancer cells in vitro.
    Cancer epidemiology, biomarkers & prevention : a publication of the American Association for Cancer Research, cosponsored by the American Society of Preventive Oncology, 2003, Volume: 12, Issue:8

    Topics: 5-alpha-Dihydroprogesterone; Animals; Cattle; Dairy Products; Estradiol; Fatty Acids; Humans; Male; Meat Products; Phytanic Acid; Prostatic Neoplasms; Racemases and Epimerases; Tumor Cells, Cultured

2003
Measurement of plasma pristanic, phytanic and very long chain fatty acids by liquid chromatography-electrospray tandem mass spectrometry for the diagnosis of peroxisomal disorders.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2003, Dec-05, Volume: 798, Issue:1

    Topics: Chromatography, High Pressure Liquid; Fatty Acids; Humans; Infant; Peroxisomal Disorders; Phytanic Acid; Spectrometry, Mass, Electrospray Ionization

2003
Biochemical markers predicting survival in peroxisome biogenesis disorders.
    Advances in experimental medicine and biology, 2003, Volume: 544

    Topics: Acyltransferases; Biomarkers; Fatty Acids; Fibroblasts; Humans; Peroxisomal Disorders; Phytanic Acid; Plasmalogens; Prognosis; Skin

2003
Identification of fatty aldehyde dehydrogenase in the breakdown of phytol to phytanic acid.
    Molecular genetics and metabolism, 2004, Volume: 82, Issue:1

    Topics: Aldehyde Oxidoreductases; Cell Culture Techniques; Fatty Acids; Fibroblasts; Gas Chromatography-Mass Spectrometry; Humans; Molecular Structure; Phytanic Acid; Phytol; Refsum Disease; Sjogren-Larsson Syndrome

2004
Hyperpipecolic acidaemia: a diagnostic tool for peroxisomal disorders.
    Molecular genetics and metabolism, 2004, Volume: 82, Issue:3

    Topics: Adolescent; Adult; Bile Acids and Salts; Chromatography; Fatty Acids; Female; France; Gas Chromatography-Mass Spectrometry; Humans; Immunoblotting; Infant; Italy; Liver; Male; Peroxisomal Disorders; Peroxisomes; Phytanic Acid; Pipecolic Acids

2004
Effect of SCP-x gene ablation on branched-chain fatty acid metabolism.
    American journal of physiology. Gastrointestinal and liver physiology, 2007, Volume: 292, Issue:3

    Topics: Acetyl-CoA C-Acetyltransferase; Alkyl and Aryl Transferases; Animals; Blotting, Western; Body Fat Distribution; Body Weight; Carrier Proteins; Fatty Acid Transport Proteins; Fatty Acids; Female; Gene Expression Regulation, Enzymologic; Lipid Metabolism; Lipids; Liver; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Mitochondria; Organ Size; Peroxisomes; Phytanic Acid; Phytol; Sex Factors

2007
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.
    The Journal of biological chemistry, 2007, Sep-14, Volume: 282, Issue:37

    Topics: Animals; Base Sequence; Coenzyme A; Fatty Acids; Male; Mice; Mice, Inbred C57BL; Molecular Sequence Data; Oxidation-Reduction; Peroxisomes; Phytanic Acid; PPAR alpha; Thiolester Hydrolases

2007
Rapid UPLC-MS/MS method for routine analysis of plasma pristanic, phytanic, and very long chain fatty acid markers of peroxisomal disorders.
    Journal of lipid research, 2008, Volume: 49, Issue:8

    Topics: Biomarkers; Chromatography, Liquid; Fatty Acids; Humans; Peroxisomal Disorders; Phytanic Acid; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2008
Phytol-induced hepatotoxicity in mice.
    Toxicologic pathology, 2009, Volume: 37, Issue:2

    Topics: Animals; Apoptosis; Body Weight; Carrier Proteins; Cell Death; Dose-Response Relationship, Drug; Fatty Acids; Female; Lipid Metabolism; Liver; Male; Mice; Mice, Inbred C57BL; Mice, Mutant Strains; Necrosis; Organ Size; Peroxisomes; Phytanic Acid; Phytol; PPAR alpha; Reference Standards; Sex Factors; Time Factors

2009
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.
    Neurobiology of disease, 2009, Volume: 36, Issue:2

    Topics: Animals; Animals, Newborn; Astrocytes; Calcium; Cells, Cultured; Fatty Acids; Hippocampus; Mitochondria; Neurons; Oligodendroglia; Phytanic Acid; Rats; Rats, Wistar; Refsum Disease

2009
An adult onset case of alpha-methyl-acyl-CoA racemase deficiency.
    Journal of inherited metabolic disease, 2010, Volume: 33 Suppl 3

    Topics: Age of Onset; Biomarkers; DNA Mutational Analysis; Fatty Acids; Genetic Predisposition to Disease; Homozygote; Humans; Leukoencephalopathies; Lipid Metabolism, Inborn Errors; Magnetic Resonance Imaging; Male; Middle Aged; Mutation; Nervous System Diseases; Phenotype; Phytanic Acid; Racemases and Epimerases; Remission Induction; Seizures; Treatment Outcome

2010
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.
    Neurobiology of disease, 2011, Volume: 43, Issue:2

    Topics: Calcium Signaling; Cell Line, Tumor; Fatty Acids; Fatty Acids, Nonesterified; HEK293 Cells; Humans; Inositol 1,4,5-Trisphosphate; Intracellular Fluid; Linoleic Acid; Lipid Metabolism; Methylamines; Phytanic Acid; Propionates; Receptors, G-Protein-Coupled; Refsum Disease

2011
[The diagnostic of peroxisomic diseases in children].
    Klinicheskaia laboratornaia diagnostika, 2012, Issue:3

    Topics: Adolescent; Adult; Child; Child, Preschool; Fatty Acids; Humans; Peroxisomal Disorders; Peroxisomes; Phytanic Acid; Reference Values

2012
Specific combination of compound heterozygous mutations in 17β-hydroxysteroid dehydrogenase type 4 (HSD17B4) defines a new subtype of D-bifunctional protein deficiency.
    Orphanet journal of rare diseases, 2012, Nov-22, Volume: 7

    Topics: 17-Hydroxysteroid Dehydrogenases; Cerebellar Ataxia; Fatty Acids; Hearing Loss, Sensorineural; Heterozygote; Hydro-Lyases; Mutation; Peroxisomal Multifunctional Protein-2; Phytanic Acid; Polyneuropathies; Retinitis Pigmentosa

2012
Serum phytanic and pristanic acid levels and prostate cancer risk in Finnish smokers.
    Cancer medicine, 2014, Volume: 3, Issue:6

    Topics: Aged; Biomarkers, Tumor; Case-Control Studies; Fatty Acids; Gas Chromatography-Mass Spectrometry; Humans; Male; Middle Aged; Phytanic Acid; Prostatic Neoplasms; Randomized Controlled Trials as Topic; Risk Factors; Smoking

2014
Effect of l-Arginine in One Patient with Peroxisome Biogenesis Disorder due to PEX12 Deficiency.
    Neuropediatrics, 2016, Volume: 47, Issue:3

    Topics: Alanine Transaminase; Arginine; Aspartate Aminotransferases; Child; Child, Preschool; Deafness; Developmental Disabilities; Fatty Acids; Female; Humans; Infant; Membrane Proteins; Muscle Hypotonia; Peroxisomal Disorders; Phytanic Acid; Pipecolic Acids; Sialorrhea

2016
A new multiplex method for the diagnosis of peroxisomal disorders allowing simultaneous determination of plasma very-long-chain fatty acids, phytanic, pristanic, docosahexaenoic and bile acids by high-performance liquid chromatography-atmospheric pressure
    Clinica chimica acta; international journal of clinical chemistry, 2016, Jul-01, Volume: 458

    Topics: Atmospheric Pressure; Bile Acids and Salts; Chromatography, High Pressure Liquid; Docosahexaenoic Acids; Fatty Acids; Humans; Peroxisomal Disorders; Phytanic Acid; Tandem Mass Spectrometry

2016
Identification and diagnostic value of phytanoyl- and pristanoyl-carnitine in plasma from patients with peroxisomal disorders.
    Molecular genetics and metabolism, 2017, Volume: 121, Issue:3

    Topics: Carnitine; Cells, Cultured; Diterpenes; Fatty Acids; Humans; Oxidation-Reduction; Peroxisomal Disorders; Phytanic Acid; Refsum Disease

2017
Phytol-induced pathology in 2-hydroxyacyl-CoA lyase (HACL1) deficient mice. Evidence for a second non-HACL1-related lyase.
    Biochimica et biophysica acta. Molecular and cell biology of lipids, 2017, Volume: 1862, Issue:9

    Topics: Animals; Disease Models, Animal; Endoplasmic Reticulum; Enoyl-CoA Hydratase; Fatty Acids; Female; Liver; Lyases; Male; Mice; Mice, Knockout; Oxidation-Reduction; Phytanic Acid; Phytol; PPAR alpha

2017