pristanic acid has been researched along with Peroxisomal Disorders in 31 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 11 (35.48) | 18.2507 |
| 2000's | 12 (38.71) | 29.6817 |
| 2010's | 8 (25.81) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Ferdinandusse, S; Herzog, K; van Lenthe, H; Vaz, FM; Wanders, RJA; Waterham, HR | 1 |
| Busanello, EN; Lobato, VG; Ribeiro, CA; Viegas, CM; Wajner, M; Zanatta, A | 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 |
| Van Veldhoven, PP | 1 |
| Horvath, J; Kapina, V; Picard, F; Sedel, F; Truffert, A; Wanders, RJ; Waterham, HR | 1 |
| Amaral, AU; Fernandes, CG; Knebel, LA; Leipnitz, G; Seminotti, B; Vargas, CR; Wajner, M; Zanatta, A | 1 |
| Mamedov, IS; Novikov, PV; Smolina, IuA; Sukhorukov, VS | 1 |
| Barth, PG; Dekker, C; Gootjes, J; Mooijer, PA; Poll-The, BT; Wanders, RJ; Waterham, HR | 2 |
| Johnson, DW; Oe, T; Trinh, MU | 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 |
| Barker, RA; Calvin, J; Ferdinandusse, S; Hogg, S; Thompson, SA; Wanders, RJ | 1 |
| Al-Dirbashi, OY; Al-Hassnan, Z; Al-Mokhadab, M; Chedrawi, A; Jacob, M; Rashed, MS; Santa, T; Shimozawa, N | 1 |
| Crane, DI; Paton, BC; Poulos, A; Sharp, PC | 1 |
| Paton, BC; Poulos, A; Sharp, PC | 1 |
| Denis, S; Jacobs, BS; Ruiter, JP; Schutgens, RB; van Roermund, CW; Wanders, RJ | 1 |
| Jakobs, C; Kulik, W; van den Heuvel, CM; Verhoeven, NM | 1 |
| Denis, S; van Grunsven, EG; van Roermund, CW; Wanders, RJ | 1 |
| van Grunsven, EG; Wanders, RJ | 1 |
| Jakobs, C; Roe, CR; Schor, DS; Verhoeven, NM; Wanders, RJ | 1 |
| Denis, S; Seedorf, U; van Berkel, E; Wanders, RJ; Wirtz, KW; Wouters, F | 1 |
| Bootsma, AH; Overmars, H; van Gennip, AH; van Lint, AE; Vreken, P; Wanders, RJ | 1 |
| Jakobs, C; Roe, CR; ten Brink, HJ; Verhoeven, NM; Wanders, RJ | 1 |
| Jakobs, C; Jansen, EE; Schor, DS; Struys, EA; Verhoeven, NM; Wanders, RJ | 1 |
| Jansen, GA; van Grunsven, EG; Wanders, RJ | 1 |
| Bennett, MJ; Brys, V; Fournier, B; Fransen, M; Mannaerts, GP; Meyhi, E; Squires, RH; Van Veldhoven, PP | 1 |
| Jakobs, C; Verhoeven, NM | 1 |
| Allen, J; Ferdinandusse, S; McLean, BN; Wanders, RJ | 1 |
| Dacremont, G; Ferdinandusse, S; Rusch, H; van Lint, AE; Vreken, P; Wanders, RJ | 1 |
4 review(s) available for pristanic acid and Peroxisomal Disorders
| Article | Year |
|---|---|
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 |
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 |
Lipid metabolism in peroxisomes: enzymology, functions and dysfunctions of the fatty acid alpha- and beta-oxidation systems in humans.
Topics: 3-Hydroxyacyl CoA Dehydrogenases; Acetyl-CoA C-Acetyltransferase; Acyl-CoA Oxidase; Cell Nucleus; Enoyl-CoA Hydratase; Fatty Acids; Gene Expression Regulation; Humans; Lipid Metabolism; Models, Biological; Oxidoreductases; Peroxisomal Disorders; Peroxisomes; Receptors, Cytoplasmic and Nuclear; Transcription Factors | 2000 |
Human metabolism of phytanic acid and pristanic acid.
Topics: Fatty Acids; Humans; Oxidation-Reduction; Peroxisomal Disorders; Peroxisomes; Phytanic Acid | 2001 |
2 trial(s) available for pristanic acid and Peroxisomal Disorders
| Article | Year |
|---|---|
Genetic heterogeneity in patients with a disorder of peroxisomal beta-oxidation: a complementation study based on pristanic acid beta-oxidation suggesting different enzyme defects.
Topics: Fatty Acids; Fibroblasts; Genetic Complementation Test; Humans; Oxidation-Reduction; Peroxisomal Disorders; Skin | 1997 |
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 |
25 other study(ies) available for pristanic acid and Peroxisomal Disorders
| Article | Year |
|---|---|
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 |
Disruption of oxidative phosphorylation and synaptic Na(+), K(+)-ATPase activity by pristanic acid in cerebellum of young rats.
Topics: Animals; Cerebellum; Disease Models, Animal; Fatty Acids; Homeostasis; Membrane Potentials; Mitochondria; Multienzyme Complexes; Oxidative Phosphorylation; Peroxisomal Disorders; Rats; Rats, Wistar; Sodium-Potassium-Exchanging ATPase; Synapses | 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 |
Relapsing rhabdomyolysis due to peroxisomal alpha-methylacyl-coa racemase deficiency.
Topics: Adult; Fatty Acids; Humans; Magnetic Resonance Imaging; Male; Peroxisomal Disorders; Racemases and Epimerases; Rhabdomyolysis; Schizophrenia; Visual Acuity | 2010 |
Pristanic acid promotes oxidative stress in brain cortex of young rats: a possible pathophysiological mechanism for brain damage in peroxisomal disorders.
Topics: Animals; Brain Chemistry; Brain Damage, Chronic; Cerebral Cortex; Disease Models, Animal; Fatty Acids; Lipid Peroxidation; Male; Oxidative Stress; Peroxisomal Disorders; Rats; Rats, Wistar | 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 |
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 |
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 |
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 |
Relapsing encephalopathy in a patient with alpha-methylacyl-CoA racemase deficiency.
Topics: Amino Acid Substitution; Brain; Brain Diseases, Metabolic, Inborn; Cerebral Cortex; Dementia; Fatty Acids; Female; Gene Expression Regulation, Enzymologic; Heredodegenerative Disorders, Nervous System; Homozygote; Humans; Magnetic Resonance Imaging; Middle Aged; Neurologic Examination; Neuropsychological Tests; Peroxisomal Disorders; Phenotype; Point Mutation; Racemases and Epimerases; Recurrence; Refsum Disease; Sequence Analysis, DNA | 2008 |
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 |
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 |
Biochemical findings in a series of Australian patients with isolated defects in peroxisomal beta-oxidation.
Topics: 3-Hydroxyacyl CoA Dehydrogenases; Australia; Enoyl-CoA Hydratase; Fatty Acids; Genetic Complementation Test; Humans; Isomerases; Microbodies; Multienzyme Complexes; Oxidation-Reduction; Peroxisomal Bifunctional Enzyme; Peroxisomal Disorders | 1996 |
Measurement of peroxisomal fatty acid beta-oxidation in cultured human skin fibroblasts.
Topics: Cell Adhesion; Cells, Cultured; Fatty Acids; Female; Fibroblasts; Humans; Indicators and Reagents; Microbodies; Oxidation-Reduction; Palmitic Acids; Peroxisomal Disorders; Pregnancy; Prenatal Diagnosis; Proteins; Skin | 1995 |
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 |
Complementation analysis of fibroblasts from peroxisomal fatty acid oxidation deficient patients shows high frequency of bifunctional enzyme deficiency plus intragenic complementation: unequivocal evidence for differential defects in the same enzyme prote
Topics: 3-Hydroxyacyl CoA Dehydrogenases; Cell Fusion; Cell Line; Enoyl-CoA Hydratase; Fatty Acids; Fibroblasts; Genetic Complementation Test; Humans; Isomerases; Multienzyme Complexes; Oxidation-Reduction; Peroxisomal Bifunctional Enzyme; Peroxisomal Disorders; Zellweger Syndrome | 1997 |
Pristanic acid beta-oxidation in peroxisomal disorders: studies in cultured human fibroblasts.
Topics: Cell Line; Cells, Cultured; Fatty Acids; Fibroblasts; Humans; Microbodies; Multienzyme Complexes; Oxidation-Reduction; Peroxisomal Disorders | 1998 |
Identification of the newly discovered 58 kDa peroxisomal thiolase SCPx as the main thiolase involved in both pristanic acid and trihydroxycholestanoic acid oxidation: implications for peroxisomal beta-oxidation disorders.
Topics: Acetyl-CoA C-Acetyltransferase; Carrier Proteins; Cholestanols; Fatty Acids; Humans; Microbodies; Oxidation-Reduction; Peroxisomal Disorders | 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 |
Analysis of pristanic acid beta-oxidation intermediates in plasma from healthy controls and patients affected with peroxisomal disorders by stable isotope dilution gas chromatography mass spectrometry.
Topics: Diagnosis, Differential; Fatty Acids; Gas Chromatography-Mass Spectrometry; Humans; Oxidation-Reduction; Oxidoreductases; Peroxisomal Disorders; Reproducibility of Results; Statistics as Topic | 1999 |
Fibroblast studies documenting a case of peroxisomal 2-methylacyl-CoA racemase deficiency: possible link between racemase deficiency and malabsorption and vitamin K deficiency.
Topics: Cells, Cultured; Fatty Acids; Female; Fibroblasts; Humans; Infant, Newborn; Isomerism; Malabsorption Syndromes; Oxidation-Reduction; Palmitic Acids; Peroxisomal Disorders; Peroxisomes; Racemases and Epimerases; Skin; Vitamin K Deficiency | 2001 |
A new defect of peroxisomal function involving pristanic acid: a case report.
Topics: Abnormalities, Multiple; Chromosome Aberrations; Fatty Acids; Genes, Recessive; Humans; Learning Disabilities; Male; Middle Aged; Peroxisomal Disorders; Racemases and Epimerases; Retinitis Pigmentosa | 2002 |
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 |