pristanic acid

pentadecanoic acid, 2,6,10,14-tetramethyl- 2,6,10,14- tetramethyl pentadecanoic acid 2,6,10,14- tetramethylpentadecanoic acid PubMed: Infantile Refsum Disease: Influence of Dietary Treatment on Plasma Phytanic Acid Levels. PubMed: Phytol is lethal for Amacr-deficient mice. PubMed: "Role of peroxisomes in human lipid metabolism and its importance for neurological development". PubMed: Rhizomelic Chondrodysplasia Punctata Type 1 Caused by a Novel Mutation in the PEX7 Gene. PubMed: Pristanic acid provokes lipid, protein, and DNA oxidative damage and reduces the antioxidant defenses in cerebellum of young rats. PubMed: A novel bile acid biosynthesis defect due to a deficiency of peroxisomal ABCD3. PubMed: Serum phytanic and pristanic acid levels and prostate cancer risk in Finnish smokers. PubMed: Disruption of oxidative phosphorylation and synaptic Na(+), K(+)-ATPase activity by pristanic acid in cerebellum of young rats. PubMed: Arginine improves peroxisome functioning in cells from patients with a mild peroxisome biogenesis disorder. PubMed: [Inborn errors of metabolism in adult neurology]. PubMed: Plasma fatty acids in premature infants with hyperbilirubinemia: before-and-after nutrition support with fish oil emulsion. PubMed: MRI characterisation of adult onset alpha-methylacyl-coA racemase deficiency diagnosed by exome sequencing. PubMed: Specific combination of compound heterozygous mutations in 17β-hydroxysteroid dehydrogenase type 4 (HSD17B4) defines a new subtype of D-bifunctional protein deficiency. PubMed: [The diagnostic of peroxisomic diseases in children]. PubMed: Experimental evidence that pristanic acid disrupts mitochondrial homeostasis in brain of young rats. PubMed: Phytanic acid metabolism in health and disease. PubMed: 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. PubMed: Mammalian peroxisomal ABC transporters: from endogenous substrates to pathology and clinical significance. PubMed: Neurochemical evidence that pristanic acid impairs energy production and inhibits synaptic Na(+), K(+)-ATPase activity in brain of young rats. PubMed: Pristanic acid promotes oxidative stress in brain cortex of young rats: a possible pathophysiological mechanism for brain damage in peroxisomal disorders. PubMed: Relapsing rhabdomyolysis due to peroxisomal alpha-methylacyl-coa racemase deficiency. PubMed: An adult onset case of alpha-methyl-acyl-CoA racemase deficiency. PubMed: Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism. PubMed: Phytanic acid--an overlooked bioactive fatty acid in dairy fat? PubMed: Induction of peroxisomal proliferator-activated receptor gamma and peroxisomal proliferator-activated receptor gamma coactivator 1 by unsaturated fatty acids, retinoic acid, and carotenoids in preadipocytes obtained from bovine white adipose tissue1,2. PubMed: 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. PubMed: Phytol-induced hepatotoxicity in mice. PubMed: Rational diagnostic strategy for Zellweger syndrome spectrum patients. PubMed: Relapsing encephalopathy in a patient with α-methylacyl-CoA racemase deficiency. PubMed: A novel Refsum-like disorder that maps to chromosome 20. PubMed: Peroxisomal acyl-CoA-oxidase deficiency: two new cases. PubMed: Rapid UPLC-MS/MS method for routine analysis of plasma pristanic, phytanic, and very long chain fatty acid markers of peroxisomal disorders. PubMed: Characterization of L-aminocarnitine, an inhibitor of fatty acid oxidation. PubMed: Relapsing encephalopathy in a patient with alpha-methylacyl-CoA racemase deficiency. PubMed: Structural and mechanistic studies on the peroxisomal oxygenase phytanoyl-CoA 2-hydroxylase (PhyH). PubMed: Peroxisomes, Refsum's disease and the alpha- and omega-oxidation of phytanic acid. PubMed: 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. PubMed: Cluster of systemic lupus erythematosus (SLE) associated with an oil field waste site: a cross sectional study. PubMed: Effect of SCP-x gene ablation on branched-chain fatty acid metabolism. PubMed: Metabolism of phytol to phytanic acid in the mouse, and the role of PPARalpha in its regulation. PubMed: Pitfall in metabolic screening in a patient with fatal peroxisomal beta-oxidation defect. PubMed: Mutations in the gene encoding peroxisomal sterol carrier protein X (SCPx) cause leukencephalopathy with dystonia and motor neuropathy. PubMed: Peroxisomal multifunctional protein 2 is essential for lipid homeostasis in Sertoli cells and male fertility in mice. PubMed: Differentiation of long-chain fatty acid oxidation disorders using alternative precursors and acylcarnitine profiling in fibroblasts. PubMed: Effect of branched-chain fatty acid on lipid dynamics in mice lacking liver fatty acid binding protein gene. PubMed: A phytol-enriched diet induces changes in fatty acid metabolism in mice both via PPARalpha-dependent and -independent pathways. PubMed: Crystal structure of 2-enoyl-CoA hydratase 2 from human peroxisomal multifunctional enzyme type 2. PubMed: Phytanic acid accumulation is associated with conduction delay and sudden cardiac death in sterol carrier protein-2/sterol carrier protein-x deficient mice. PubMed: Tremor and deep white matter changes in alpha-methylacyl-CoA racemase deficiency. PubMed: Hyperpipecolic acidaemia: a diagnostic tool for peroxisomal disorders. PubMed: Identification of fatty aldehyde dehydrogenase in the breakdown of phytol to phytanic acid. PubMed: Molecular cloning and characterization of two mouse peroxisome proliferator-activated receptor alpha (PPARalpha)-regulated peroxisomal acyl-CoA thioesterases. PubMed: Sexually dimorphic metabolism of branched-chain lipids in C57BL/6J mice. PubMed: Phytanic and pristanic acid are naturally occuring ligands. PubMed: Biochemical markers predicting survival in peroxisome biogenesis disorders. PubMed: Measurement of plasma pristanic, phytanic and very long chain fatty acids by liquid chromatography-electrospray tandem mass spectrometry for the diagnosis of peroxisomal disorders. PubMed: Novel mutations in the PEX12 gene of patients with a peroxisome biogenesis disorder. PubMed: Branched fatty acids in dairy and beef products markedly enhance alpha-methylacyl-CoA racemase expression in prostate cancer cells in vitro. PubMed: Membrane differential filtration is safe and effective for the long-term treatment of Refsum syndrome--an update of treatment modalities and pathophysiological cognition. PubMed: The chemical biology of branched-chain lipid metabolism. PubMed: Liver disease caused by failure to racemize trihydroxycholestanoic acid: gene mutation and effect of bile acid therapy. PubMed: Biochemical markers predicting survival in peroxisome biogenesis disorders. PubMed: Branched chain fatty acids induce nitric oxide-dependent apoptosis in vascular smooth muscle cells. PubMed: Phytanic acid, but not pristanic acid, mediates the positive effects of phytol derivatives on brown adipocyte differentiation. PubMed: Stereochemistry of the peroxisomal branched-chain fatty acid alpha- and beta-oxidation systems in patients suffering from different peroxisomal disorders. PubMed: A new defect of peroxisomal function involving pristanic acid: a case report. PubMed: Demonstration of dimethylnonanoyl-CoA thioesterase activity in rat liver peroxisomes followed by purification and molecular cloning of the thioesterase involved. PubMed: Characterization of an acyl-coA thioesterase that functions as a major regulator of peroxisomal lipid metabolism. PubMed: Human metabolism of phytanic acid and pristanic acid. PubMed: Fibroblast studies documenting a case of peroxisomal 2-methylacyl-CoA racemase deficiency: possible link between racemase deficiency and malabsorption and vitamin K deficiency. PubMed: Clinical consequences of defects in peroxisomal beta-oxidation. PubMed: Further insights into peroxisomal lipid breakdown via alpha- and beta-oxidation. PubMed: Identification of pristanal dehydrogenase activity in peroxisomes: conclusive evidence that the complete phytanic acid alpha-oxidation pathway is localized in peroxisomes. PubMed: Peroxisomal lipid degradation via beta- and alpha-oxidation in mammals. PubMed: Plasma analysis of di- and trihydroxycholestanoic acid diastereoisomers in peroxisomal alpha-methylacyl-CoA racemase deficiency. PubMed: Subcellular localization and physiological role of alpha-methylacyl-CoA racemase. PubMed: Pristanic acid and phytanic acid: naturally occurring ligands for the nuclear receptor peroxisome proliferator-activated receptor alpha. PubMed: Mitochondrial and peroxisomal targeting of 2-methylacyl-CoA racemase in humans. PubMed: Lipid metabolism in peroxisomes: enzymology, functions and dysfunctions of the fatty acid alpha- and beta-oxidation systems in humans. PubMed: Role and organization of peroxisomal beta-oxidation. PubMed: Mutations in the gene encoding peroxisomal alpha-methylacyl-CoA racemase cause adult-onset sensory motor neuropathy. PubMed: Aberrant oxidation of the cholesterol side chain in bile acid synthesis of sterol carrier protein-2/sterol carrier protein-x knockout mice. PubMed: Phytanic acid alpha-oxidation: identification of 2-hydroxyphytanoyl-CoA lyase in rat liver and its localisation in peroxisomes. PubMed: Molecular cloning and expression of human carnitine octanoyltransferase: evidence for its role in the peroxisomal beta-oxidation of branched-chain fatty acids. PubMed: Peroxisomal beta-oxidation enzymes. PubMed: Human very-long-chain acyl-CoA synthetase: cloning, topography, and relevance to branched-chain fatty acid metabolism. PubMed: 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. PubMed: Peroxisomal bifunctional protein deficiency revisited: resolution of its true enzymatic and molecular basis. PubMed: Studies on the oxidation of phytanic acid and pristanic acid in human fibroblasts by acylcarnitine analysis. PubMed: The metabolism of phytanic acid and pristanic acid in man: a review. PubMed: Defective peroxisome biogenesis with a neuromuscular disorder resembling Werdnig-Hoffmann disease. PubMed: New insights in peroxisomal beta-oxidation. Implications for human peroxisomal disorders. PubMed: Rapid stable isotope dilution analysis of very-long-chain fatty acids, pristanic acid and phytanic acid using gas chromatography-electron impact mass spectrometry. PubMed: 2,6-Dimethylheptanoyl-CoA is a specific substrate for long-chain acyl-CoA dehydrogenase (LCAD): evidence for a major role of LCAD in branched-chain fatty acid oxidation. PubMed: 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. PubMed: Involvement of microsomal fatty aldehyde dehydrogenase in the alpha-oxidation of phytanic acid. PubMed: Pristanic acid beta-oxidation in peroxisomal disorders: studies in cultured human fibroblasts. PubMed: Niemann-Pick disease type C and defective peroxisomal beta-oxidation of branched-chain substrates. PubMed: Human long chain, very long chain and medium chain acyl-CoA dehydrogenases are specific for the S-enantiomer of 2- methylpentadecanoyl-CoA. PubMed: Peroxisomal D-hydroxyacyl-CoA dehydrogenase deficiency: resolution of the enzyme defect and its molecular basis in bifunctional protein deficiency. PubMed: Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts. PubMed: Phytanic acid alpha-oxidation in peroxisomal disorders: studies in cultured human fibroblasts. PubMed: Phytanic acid alpha-oxidation: decarboxylation of 2-hydroxyphytanoyl-CoA to pristanic acid in human liver. PubMed: A new peroxisomal beta-oxidation disorder in twin neonates: defective oxidation of both cerotic and pristanic acids. PubMed: Resolution of the phytanic acid alpha-oxidation pathway: identification of pristanal as product of the decarboxylation of 2-hydroxyphytanoyl-CoA. PubMed: Stable isotope studies of phytanic acid alpha-oxidation: in vivo production of formic acid. PubMed: Sterol carrier protein X (SCPx) is a peroxisomal branched-chain beta-ketothiolase specifically reacting with 3-oxo-pristanoyl-CoA: a new, unique role for SCPx in branched-chain fatty acid metabolism in peroxisomes. PubMed: Evidence that multifunctional protein 2, and not multifunctional protein 1, is involved in the peroxisomal beta-oxidation of pristanic acid. PubMed: 2-methylacyl racemase: a coupled assay based on the use of pristanoyl-CoA oxidase/peroxidase and reinvestigation of its subcellular distribution in rat and human liver. PubMed: Formation of 2,3-pristenic acid and 3-hydroxypristanic acid from pristanic acid in human liver. PubMed: Genetic heterogeneity in patients with a disorder of peroxisomal beta-oxidation: a complementation study based on pristanic acid beta-oxidation suggesting different enzyme defects. PubMed: 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 protein. PubMed: Rhizomelic chondrodysplasia punctata-like phenotype in a newborn male with normal peroxisomal function. PubMed: Biochemical findings in a series of Australian patients with isolated defects in peroxisomal beta-oxidation. PubMed: Studies on the degradation of [U-3H]-phytanic acid and [U-3H]-pristanic acid in cultured fibroblasts from children with peroxisomal disorders. PubMed: Oxidation of pristanic acid in fibroblasts and its application to the diagnosis of peroxisomal beta-oxidation defects. PubMed: Ataxia associated with increased plasma concentrations of pristanic acid, phytanic acid and C27 bile acids but normal fibroblast branched-chain fatty acid oxidation. PubMed: Impaired peroxisomal fatty acid oxidation in human skin fibroblasts with a mitochondrial acylcarnitine/carnitine translocase deficiency. PubMed: Ketoconazole and other imidazole derivatives are potent inhibitors of peroxisomal phytanic acid alpha-oxidation. PubMed: The role of peroxisomes in intermediary metabolism. PubMed: Fatty acid beta-oxidation in peroxisomes and mitochondria: the first, unequivocal evidence for the involvement of carnitine in shuttling propionyl-CoA from peroxisomes to mitochondria. PubMed: Phytanic acid oxidation: topographical localization of phytanoyl-CoA ligase and transport of phytanic acid into human peroxisomes. PubMed: Substrate specificity of rat liver mitochondrial carnitine palmitoyl transferase I: evidence against alpha-oxidation of phytanic acid in rat liver mitochondria. PubMed: Measurement of very long-chain fatty acids, phytanic and pristanic acid in plasma and cultured fibroblasts by gas chromatography. PubMed: Pre- and postnatal diagnosis of peroxisomal disorders using stable-isotope dilution gas chromatography--mass spectrometry. PubMed: Activity measurements of acyl-CoA oxidases in human liver. PubMed: Measurement of peroxisomal fatty acid beta-oxidation in cultured human skin fibroblasts. PubMed: Phytanic acid alpha-oxidation in rat liver mitochondria. PubMed: 2-Hydroxyphytanic acid oxidase activity in rat and human liver and its deficiency in the Zellweger syndrome. PubMed: A new peroxisomal disorder with fetal and neonatal adrenal insufficiency. PubMed: 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. PubMed: Heterogeneity in di/trihydroxycholestanoic acidaemia. PubMed: Beta-oxidation of fatty acids in cultured human skin fibroblasts devoid of the capacity for oxidative phosphorylation. PubMed: Clinical and biochemical characteristics of peroxisomal disorders: an update. PubMed: Phytanic acid alpha-oxidase deficiency (Refsum disease) presenting in infancy. PubMed: Intraorganellar localization of CoASH-independent phytanic acid oxidation in human liver peroxisomes. PubMed: A new peroxisomal disease with impaired phytanic and pipecolic acid oxidation. PubMed: Refsum disease: a defect in the alpha-oxidation of phytanic acid in peroxisomes. PubMed: Diagnosis of peroxisomal disorders by analysis of phytanic and pristanic acids in stored blood spots collected at neonatal screening. PubMed: Phytanic acid alpha-oxidation. Differential subcellular localization in rat and human tissues and its inhibition by nycodenz. PubMed: The CoA esters of 2-methyl-branched chain fatty acids and of the bile acid intermediates di- and trihydroxycoprostanic acids are oxidized by one single peroxisomal branched chain acyl-CoA oxidase in human liver and kidney. PubMed: The deficient degradation of synthetic 2- and 3-methyl-branched fatty acids in fibroblasts from patients with peroxisomal disorders. PubMed: [Zellweger syndrome, neonatal adrenoleukodystrophy or infantile Refsum's disease in a case with generalized peroxisome defect?]. PubMed: Phytanic acid alpha-oxidation in human cultured skin fibroblasts. PubMed: Peroxisomal beta-oxidation of branched chain fatty acids in human skin fibroblasts. PubMed: In vivo study of phytanic acid alpha-oxidation in classic Refsum's disease and chondrodysplasia punctata. PubMed: Phytanic acid alpha-oxidation: accumulation of 2-hydroxyphytanic acid and absence of 2-oxophytanic acid in plasma from patients with peroxisomal disorders. PubMed: Characteristics and subcellular localization of pristanoyl-CoA synthetase in rat liver. PubMed: Molecular analysis of disorders of peroxisomal beta-oxidation. PubMed: Activation and oxidation of pristanic acid in rat liver: identification of a distinct, clofibrate non-inducible pristanoyl-CoA oxidase. PubMed: Autopsy findings in two siblings with infantile Refsum disease. PubMed: Pristanic acid and phytanic acid in plasma from patients with peroxisomal disorders: stable isotope dilution analysis with electron capture negative ion mass fragmentography. PubMed: Mitochondrial and peroxisomal beta oxidation of the branched chain fatty acid 2-methylpalmitate in rat liver. PubMed: Identification and purification of a peroxisomal branched chain fatty acyl-CoA oxidase. PubMed: Separation of phytanic and pristanic acid by high-pressure liquid chromatography: application of the method. PubMed: Pristanic acid does not accumulate in peroxisomal acyl-CoA oxidase deficiency: evidence for a distinct peroxisomal pristanyl-CoA oxidase. PubMed: Pristanic acid and phytanic acid in plasma from patients with a single peroxisomal enzyme deficiency. PubMed: Identification of pristanoyl-CoA oxidase activity in human liver and its deficiency in the Zellweger syndrome. PubMed: Defective oxidation of pristanic acid by fibroblasts from patients with disorders in propionic acid metabolism. PubMed: Metabolism of branched chain fatty acids in peroxisomal disorders. PubMed: Phytanic acid, pristanic acid, and very-long-chain fatty acid methyl esters measured simultaneously by capillary gas chromatography. PubMed: Disposition and metabolism of pristane in rat. PubMed: Accumulation of pristanic acid (2, 6, 10, 14 tetramethylpentadecanoic acid) in the plasma of patients with generalised peroxisomal dysfunction. PubMed: Phytanic acid oxidase activity in cultured skin fibroblasts. Diagnostic usefulness and limitations. PubMed: 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. PubMed: Metabolism of long-chain isoprenoid alcohols. Incorporation of phytol and dihydrophytol into the lipids of rat brain. PubMed: Localization of the oxidative defect in phytanic acid degradation in patients with Refsum's disease. PubMed: Separation of lipids containing phytanic acid by thin-layer chromatography. PubMed: Isoprenoid acids in recent sediments. PubMed: The occurrence of diastereomers of phytanic and pristanic acids and their determination by gas-liquid chromatography. PubMed: Refsum's disease: nature of the enzyme defect. PubMed: Pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) and phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) content of human and animal tissues. PubMed: Synthesis of 2,6,10,14-tetramethylpentadecanoic acid (pristanic acid). PubMed: Occurrence of 2,6,10,14-tetramethylpentadecanoic acid in sheep fat. PubMed: The isolation and identification of 2,6,10,14-tetramethylpentadecanoic acid from butterfat.