pristanic acid has been researched along with phytanic acid in 64 studies
Studies (pristanic acid) | Trials (pristanic acid) | Recent Studies (post-2010) (pristanic acid) | Studies (phytanic acid) | Trials (phytanic acid) | Recent Studies (post-2010) (phytanic acid) |
---|---|---|---|---|---|
103 | 2 | 17 | 435 | 3 | 70 |
Timeframe | Studies, this research(%) | All Research% |
---|---|---|
pre-1990 | 3 (4.69) | 18.7374 |
1990's | 32 (50.00) | 18.2507 |
2000's | 18 (28.13) | 29.6817 |
2010's | 10 (15.63) | 24.3611 |
2020's | 1 (1.56) | 2.80 |
Authors | Studies |
---|---|
Hancock, AJ; Kates, M | 1 |
Jakobs, C; Kneer, J; Kok, RM; Poll-The, BT; Saudubray, JM; Schor, DS; Stellaard, F; ten Brink, HJ | 1 |
Jakobs, C; Kok, RM; Schor, DS; Stellaard, F; ten Brink, HJ; van den Heuvel, CM; Wanders, RJ | 1 |
Chow, CW; Christodoulou, J; Danks, DM; Fellenberg, AJ; Poulos, A | 1 |
Jakobs, C; Kok, RM; Poll-The, BT; Schor, DS; ten Brink, HJ; Wanders, RJ | 1 |
Kalipada, P; Lazo, O; Singh, AK; Singh, I | 1 |
Brogan, M; Johnson, D; Poulos, A; Singh, H | 1 |
Jakobs, C; Schutgens, RB; Stellaard, F; ten Brink, HJ; Wanders, RJ | 1 |
Jakobs, C; Poll-The, BT; Saudubray, JM; ten Brink, HJ; Wanders, RJ | 1 |
Kase, BF; Olund, J; Sisfontes, L | 1 |
Fellenberg, AJ; Johnson, DW; Poulos, A; Sharp, P | 1 |
Applegarth, DA; Clarke, LA; Harris, HM; Wong, J | 1 |
Barbosa, E; Pahan, K; Singh, AK; Singh, I | 1 |
Molzer, B; Schmitt, K; Stöckler, S; Tulzer, G; Tulzer, W | 1 |
Eyssen, HJ; Huang, S; Mannaerts, GP; Van Veldhoven, PP | 1 |
Gulati, S; Pahan, K; Singh, I | 1 |
Poulos, A; Singh, H | 1 |
Brandt, NJ; Christensen, E; Jakobs, C; ten Brink, HJ; Wanders, RJ | 1 |
Beckman, K; Poulos, A; Singh, H | 1 |
Christensen, E; Jakobs, C; Largillière, C; ten Brink, HJ; van den Heuvel, CM | 1 |
Crane, DI; Paton, BC; Poulos, A; Sharp, PC | 1 |
Björkhem, I; Kase, BF | 1 |
Casteels, M; Clayton, PT; Johnson, AW; Lynes, GW; Mannaerts, G; Mills, KA; Wilson, J | 1 |
Lima, MR; Mota, CR; Nogueira, A; Vilarinho, A | 1 |
Jakobs, C; Kulik, W; van den Heuvel, CM; Verhoeven, NM | 1 |
Cocquyt, G; Dacremont, G; Vincent, G | 1 |
Jakobs, C; Schor, DS; ten Brink, HJ; Verhoeven, NM; Wanders, RJ | 1 |
Christensen, E; Jakobs, C; Leth, H; Pedersen, SA; Schutgens, RB; Wanders, RJ | 1 |
Jakobs, C; Jansen, GA; Schor, DS; Verhoeven, NM; Wanders, RJ | 1 |
Jakobs, C; Kok, RM; Roe, CR; Roe, DS; Verhoeven, NM; Wanders, RJ | 1 |
Carney, G; Jakobs, C; Rizzo, WB; Somers, MP; Verhoeven, NM; Wanders, RJ | 1 |
Bootsma, AH; Overmars, H; van Gennip, AH; van Lint, AE; Vreken, P; Wanders, RJ | 1 |
Jakobs, C; Poll-The, BT; Saudubray, JM; Verhoeven, NM; Wanders, RJ | 1 |
Jakobs, C; Roe, CR; ten Brink, HJ; Verhoeven, NM; Wanders, RJ | 1 |
Kim, DG; Mihalik, SJ; Steinberg, SJ; Wang, SJ; Watkins, PA | 1 |
Jansen, GA; Poll-The, BT; van Der Burg, B; van Der Saag, PT; Wanders, RJ; Zomer, AW | 1 |
Jakobs, C; Verhoeven, NM | 1 |
Dacremont, G; Ferdinandusse, S; Rusch, H; van Lint, AE; Vreken, P; Wanders, RJ | 1 |
Giralt, M; Iglesias, R; Schluter, A; Villarroya, F | 1 |
Barth, PG; Dekker, C; Gootjes, J; Mooijer, PA; Poll-The, BT; Wanders, RJ; Waterham, HR | 2 |
Gäckler, D; Kingreen, H; Klingel, R; Muselmann, L; Straube, R; Thiele, A | 1 |
Evans, J; Ho, SM; Jiang, Z; L'Esperance, BS; Lam, YW; Leav, I; Mobley, JA; Wotkowitz, C; Zielie, P | 1 |
Johnson, DW; Oe, T; Trinh, MU | 1 |
Poll-The, BT; van der Saag, PT; Zomer, AW | 1 |
Dacremont, G; Jansen, GA; Rontani, JF; van den Brink, DM; van Miert, JN; Wanders, RJ | 1 |
Baumgartner, MR; Bonetti, G; Jakobs, C; Nassogne, MC; Peduto, A; Poll-The, BT; Rabier, D; Saudubray, JM; Spada, M; Verhoeven, NM | 1 |
Alger, S; Burdon, MA; Chavda, S; Clarke, CE; Denis, S; Ferdinandusse, S; Preece, MA; Wanders, RJ | 1 |
Atshaves, BP; Ball, J; Kier, AB; Landrock, D; Mackie, JT; Maeda, N; McIntosh, AL; Payne, HR; Schroeder, F | 1 |
Alexson, SEH; Hunt, MC; Westin, MAK | 1 |
Al-Dirbashi, OY; Al-Hassnan, Z; Al-Mokhadab, M; Chedrawi, A; Jacob, M; Rashed, MS; Santa, T; Shimozawa, N | 1 |
Atshaves, BP; Kier, AB; Mackie, JT; McIntosh, AL; Payne, HR; Schroeder, F | 1 |
Kahlert, S; Kruska, N; Reiser, G; Rönicke, S | 1 |
Van Veldhoven, PP | 1 |
Freeman, WD; Gavrilov, DK; Matern, D; Oglesbee, D; Smith, EH; Tortorelli, S; Vavra, MW | 1 |
Kruska, N; Reiser, G | 1 |
Mamedov, IS; Novikov, PV; Smolina, IuA; Sukhorukov, VS | 1 |
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, T | 1 |
Albanes, D; Gann, PH; Männistö, S; Moser, AB; Snyder, K; Weinstein, SJ; Wright, ME | 1 |
Briand, G; Cheillan, D; Feillet, F; Montaut-Verient, B; Schmitt, E; Sorlin, A; Wiedemann, A | 1 |
Antonetti, G; Bertini, E; Boenzi, S; Cappa, M; Dionisi-Vici, C; Rizzo, C; Semeraro, M | 1 |
Ferdinandusse, S; Herzog, K; van Lenthe, H; Vaz, FM; Wanders, RJA; Waterham, HR | 1 |
Asselberghs, S; Baes, M; De Schryver, E; Meyhi, E; Mezzar, S; Morvay, PL; Van Veldhoven, PP | 1 |
Bobe, G; Garzotto, M; Kopp, R; Shannon, J; Takata, Y; Zhang, Z | 1 |
6 review(s) available for pristanic acid and phytanic acid
Article | Year |
---|---|
The metabolism of phytanic acid and pristanic acid in man: a review.
Topics: Animals; Fatty Acids; Humans; Oxidation-Reduction; Phytanic Acid | 1998 |
Human metabolism of phytanic acid and pristanic acid.
Topics: Fatty Acids; Humans; Oxidation-Reduction; Peroxisomal Disorders; Peroxisomes; Phytanic Acid | 2001 |
Phytanic and pristanic acid are naturally occuring ligands.
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.
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.
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.
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 |
1 trial(s) available for pristanic acid and phytanic acid
Article | Year |
---|---|
Rapid stable isotope dilution analysis of very-long-chain fatty acids, pristanic acid and phytanic acid using gas chromatography-electron impact mass spectrometry.
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 |
57 other study(ies) available for pristanic acid and phytanic acid
Article | Year |
---|---|
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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?].
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Topics: Biomarkers; Chromatography, Liquid; Fatty Acids; Humans; Peroxisomal Disorders; Phytanic Acid; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry | 2008 |
Phytol-induced hepatotoxicity in mice.
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.
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.
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.
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].
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.
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.
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.
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
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.
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.
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 |