Page last updated: 2024-09-03

pristanic acid and Cerebro-Hepato-Renal Syndrome

pristanic acid has been researched along with Cerebro-Hepato-Renal Syndrome in 11 studies

Research

Studies (11)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's10 (90.91)18.2507
2000's1 (9.09)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Barth, PG; Dekker, C; Gootjes, J; Mooijer, PA; Poll-The, BT; Wanders, RJ; Waterham, HR1
Molzer, B; Schmitt, K; Stöckler, S; Tulzer, G; Tulzer, W1
Eyssen, HJ; Huang, S; Mannaerts, GP; Van Veldhoven, PP1
Crane, DI; Paton, BC; Poulos, A; Sharp, PC1
Björkhem, I; Kase, BF1
Denis, S; van Grunsven, EG; van Roermund, CW; Wanders, RJ1
Jakobs, C; Jansen, GA; Kok, RM; 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
Johnson, D; Poulos, A; Singh, H; Usher, S1

Other Studies

11 other study(ies) available for pristanic acid and Cerebro-Hepato-Renal Syndrome

ArticleYear
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
[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
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
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
    Biochemical and biophysical research communications, 1997, Jun-09, Volume: 235, Issue:1

    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
Formation of 2,3-pristenic acid and 3-hydroxypristanic acid from pristanic acid in human liver.
    Journal of inherited metabolic disease, 1997, Volume: 20, Issue:3

    Topics: Adenosine Triphosphate; Coenzyme A; Fatty Acids; Fatty Acids, Unsaturated; Humans; In Vitro Techniques; Liver; Magnesium; Oxidation-Reduction; Zellweger Syndrome

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
Metabolism of branched chain fatty acids in peroxisomal disorders.
    Journal of inherited metabolic disease, 1990, Volume: 13, Issue:3

    Topics: Adrenoleukodystrophy; Cells, Cultured; Chondrodysplasia Punctata; Fatty Acids; Fibroblasts; Heptanoic Acids; Humans; Metabolism, Inborn Errors; Microbodies; Oxidation-Reduction; Refsum Disease; Stearic Acids; Zellweger Syndrome

1990