| MeSH term | MeSH ID | Detail |
|---|---|---|
| Body Weight | D001835 | 333 associated lipids |
| Abnormalities, Multiple | D000015 | 13 associated lipids |
| Adrenoleukodystrophy | D000326 | 29 associated lipids |
| Refsum Disease | D012035 | 19 associated lipids |
| Cholestasis | D002779 | 23 associated lipids |
| Lipid Metabolism, Inborn Errors | D008052 | 26 associated lipids |
| Brain Diseases, Metabolic, Inborn | D020739 | 10 associated lipids |
| Peroxisomal Disorders | D018901 | 5 associated lipids |
| Brain Damage, Chronic | D001925 | 6 associated lipids |
| Chondrodysplasia Punctata | D002806 | 8 associated lipids |
Pristanic acid
Pristanic acid is a lipid of Prenol Lipids (PR) class. Pristanic acid is associated with abnormalities such as Refsum Disease, Peroxisomal Disorders, Hereditary Diseases, Peripheral Neuropathy and Sensory neuropathy. The involved functions are known as physiological aspects, Regulation, Pathogenesis, Oxidation and Peroxisome Proliferator-activated Receptor Activity [MoA]. Pristanic acid often locates in peroxisome, Body tissue, Mitochondria, Membrane of peroxisome and Organelles. The associated genes with Pristanic acid are PSG5 gene, LGALS4 gene, PEX2 gene, ACSL4 gene and ACSL1 Gene. The related lipids are pristanic acid, Fatty Acids, branched chain fatty acid, Plasmalogens and 3-hydroxypristanic acid.
Cross Reference
Introduction
To understand associated biological information of Pristanic acid, we collected biological information of abnormalities, associated pathways, cellular/molecular locations, biological functions, related genes/proteins, lipids and common seen animal/experimental models with organized paragraphs from literatures.
What diseases are associated with Pristanic acid?
Pristanic acid is suspected in Peroxisomal Disorders, Refsum Disease, Protein Deficiency, Retinitis Pigmentosa, Enzyme Deficiency, Hereditary Diseases and other diseases in descending order of the highest number of associated sentences.
Related references are mostly published in these journals:
| Disease | Cross reference | Weighted score | Related literature |
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Possible diseases from mapped MeSH terms on references
We collected disease MeSH terms mapped to the references associated with Pristanic acid
PubChem Associated disorders and diseases
What pathways are associated with Pristanic acid
Lipid pathways are not clear in current pathway databases. We organized associated pathways with Pristanic acid through full-text articles, including metabolic pathways or pathways of biological mechanisms.
Related references are published most in these journals:
| Pathway name | Related literatures |
|---|
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PubChem Biomolecular Interactions and Pathways
Link to PubChem Biomolecular Interactions and PathwaysWhat cellular locations are associated with Pristanic acid?
Visualization in cellular structure
Associated locations are in red color. Not associated locations are in black.
Related references are published most in these journals:
| Location | Cross reference | Weighted score | Related literatures |
|---|
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What functions are associated with Pristanic acid?
Related references are published most in these journals:
| Function | Cross reference | Weighted score | Related literatures |
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What lipids are associated with Pristanic acid?
Related references are published most in these journals:
| Lipid concept | Cross reference | Weighted score | Related literatures |
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What genes are associated with Pristanic acid?
Related references are published most in these journals:
| Gene | Cross reference | Weighted score | Related literatures |
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What common seen animal models are associated with Pristanic acid?
There are no associated biomedical information in the current reference collection.
NCBI Entrez Crosslinks
All references with Pristanic acid
Download all related citations| Authors | Title | Published | Journal | PubMed Link |
|---|---|---|---|---|
| Clayton PT et al. | Ataxia associated with increased plasma concentrations of pristanic acid, phytanic acid and C27 bile acids but normal fibroblast branched-chain fatty acid oxidation. | 1996 | J. Inherit. Metab. Dis. | pmid:8982949 |
| Dieuaide-Noubhani M et al. | Evidence that multifunctional protein 2, and not multifunctional protein 1, is involved in the peroxisomal beta-oxidation of pristanic acid. | 1997 | Biochem. J. | pmid:9230115 |
| van Grunsven EG et al. | 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. | 1997 | Biochem. Biophys. Res. Commun. | pmid:9196058 |
| Mota CR et al. | Rhizomelic chondrodysplasia punctata-like phenotype in a newborn male with normal peroxisomal function. | 1997 | J Pediatr Orthop B | pmid:9039662 |
| van Grunsven EG and Wanders RJ | Genetic heterogeneity in patients with a disorder of peroxisomal beta-oxidation: a complementation study based on pristanic acid beta-oxidation suggesting different enzyme defects. | 1997 | J. Inherit. Metab. Dis. | pmid:9266375 |
| Verhoeven NM et al. | Formation of 2,3-pristenic acid and 3-hydroxypristanic acid from pristanic acid in human liver. | 1997 | J. Inherit. Metab. Dis. | pmid:9266376 |
| Verhoeven NM et al. | Phytanic acid alpha-oxidation: decarboxylation of 2-hydroxyphytanoyl-CoA to pristanic acid in human liver. | 1997 | J. Lipid Res. | pmid:9374128 |
| Verhoeven NM et al. | Resolution of the phytanic acid alpha-oxidation pathway: identification of pristanal as product of the decarboxylation of 2-hydroxyphytanoyl-CoA. | 1997 | Biochem. Biophys. Res. Commun. | pmid:9266824 |
| Verhoeven NM et al. | Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts. | 1998 | J. Lipid Res. | pmid:9469587 |
| Verhoeven NM et al. | Pristanic acid beta-oxidation in peroxisomal disorders: studies in cultured human fibroblasts. | 1998 | Biochim. Biophys. Acta | pmid:9555092 |
| Vreken P et al. | Rapid stable isotope dilution analysis of very-long-chain fatty acids, pristanic acid and phytanic acid using gas chromatography-electron impact mass spectrometry. | 1998 | J. Chromatogr. B Biomed. Sci. Appl. | pmid:9746242 |
| Wanders RJ et al. | 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. | 1998 | J. Inherit. Metab. Dis. | pmid:9686381 |
| Sequeira JS et al. | Niemann-Pick disease type C and defective peroxisomal beta-oxidation of branched-chain substrates. | 1998 | J. Inherit. Metab. Dis. | pmid:9584266 |
| Verhoeven NM et al. | The metabolism of phytanic acid and pristanic acid in man: a review. | 1998 | J. Inherit. Metab. Dis. | pmid:9819701 |
| Verhoeven NM et al. | Studies on the oxidation of phytanic acid and pristanic acid in human fibroblasts by acylcarnitine analysis. | 1998 | J. Inherit. Metab. Dis. | pmid:9819705 |
| Verhoeven NM et al. | Involvement of microsomal fatty aldehyde dehydrogenase in the alpha-oxidation of phytanic acid. | 1998 | FEBS Lett. | pmid:9662422 |
| Steinberg SJ et al. | Human very-long-chain acyl-CoA synthetase: cloning, topography, and relevance to branched-chain fatty acid metabolism. | 1999 | Biochem. Biophys. Res. Commun. | pmid:10198260 |
| van Grunsven EG et al. | Peroxisomal bifunctional protein deficiency revisited: resolution of its true enzymatic and molecular basis. | 1999 | Am. J. Hum. Genet. | pmid:9915948 |
| Ferdinandusse S et al. | Molecular cloning and expression of human carnitine octanoyltransferase: evidence for its role in the peroxisomal beta-oxidation of branched-chain fatty acids. | 1999 | Biochem. Biophys. Res. Commun. | pmid:10486279 |
| Verhoeven NM et al. | 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. | 1999 | J. Lipid Res. | pmid:9925655 |
| Wanders RJ et al. | Lipid metabolism in peroxisomes: enzymology, functions and dysfunctions of the fatty acid alpha- and beta-oxidation systems in humans. | 2000 | Biochem. Soc. Trans. | pmid:10816116 |
| Zomer AW et al. | Pristanic acid and phytanic acid: naturally occurring ligands for the nuclear receptor peroxisome proliferator-activated receptor alpha. | 2000 | J. Lipid Res. | pmid:11060349 |
| Ferdinandusse S et al. | Subcellular localization and physiological role of alpha-methylacyl-CoA racemase. | 2000 | J. Lipid Res. | pmid:11060359 |
| Ferdinandusse S et al. | Plasma analysis of di- and trihydroxycholestanoic acid diastereoisomers in peroxisomal alpha-methylacyl-CoA racemase deficiency. | 2001 | J. Lipid Res. | pmid:11160375 |
| Van Veldhoven PP et al. | Fibroblast studies documenting a case of peroxisomal 2-methylacyl-CoA racemase deficiency: possible link between racemase deficiency and malabsorption and vitamin K deficiency. | 2001 | Eur. J. Clin. Invest. | pmid:11473573 |
| Verhoeven NM and Jakobs C | Human metabolism of phytanic acid and pristanic acid. | 2001 | Prog. Lipid Res. | pmid:11591435 |
| Jansen GA et al. | Identification of pristanal dehydrogenase activity in peroxisomes: conclusive evidence that the complete phytanic acid alpha-oxidation pathway is localized in peroxisomes. | 2001 | Biochem. Biophys. Res. Commun. | pmid:11341778 |
| Schluter A et al. | Phytanic acid, but not pristanic acid, mediates the positive effects of phytol derivatives on brown adipocyte differentiation. | 2002 | FEBS Lett. | pmid:12062414 |
| Idel S et al. | Branched chain fatty acids induce nitric oxide-dependent apoptosis in vascular smooth muscle cells. | 2002 | J. Biol. Chem. | pmid:12368296 |
| Hunt MC et al. | Characterization of an acyl-coA thioesterase that functions as a major regulator of peroxisomal lipid metabolism. | 2002 | J. Biol. Chem. | pmid:11673457 |
| Ferdinandusse S et al. | Stereochemistry of the peroxisomal branched-chain fatty acid alpha- and beta-oxidation systems in patients suffering from different peroxisomal disorders. | 2002 | J. Lipid Res. | pmid:11893780 |
| McLean BN et al. | A new defect of peroxisomal function involving pristanic acid: a case report. | 2002 | J. Neurol. Neurosurg. Psychiatr. | pmid:11861706 |
| Ofman R et al. | Demonstration of dimethylnonanoyl-CoA thioesterase activity in rat liver peroxisomes followed by purification and molecular cloning of the thioesterase involved. | 2002 | Biochem. Biophys. Res. Commun. | pmid:11785945 |
| Gootjes J et al. | Biochemical markers predicting survival in peroxisome biogenesis disorders. | 2002 | Neurology | pmid:12473763 |
| Setchell KD et al. | Liver disease caused by failure to racemize trihydroxycholestanoic acid: gene mutation and effect of bile acid therapy. | 2003 | Gastroenterology | pmid:12512044 |
| Straube R et al. | Membrane differential filtration is safe and effective for the long-term treatment of Refsum syndrome--an update of treatment modalities and pathophysiological cognition. | 2003 | Transfus. Apher. Sci. | pmid:12877898 |
| Mobley JA et al. | Branched fatty acids in dairy and beef products markedly enhance alpha-methylacyl-CoA racemase expression in prostate cancer cells in vitro. | 2003 | Cancer Epidemiol. Biomarkers Prev. | pmid:12917210 |
| Gootjes J et al. | Biochemical markers predicting survival in peroxisome biogenesis disorders. | 2003 | Adv. Exp. Med. Biol. | pmid:14713214 |
| Zomer AW et al. | Phytanic and pristanic acid are naturally occuring ligands. | 2003 | Adv. Exp. Med. Biol. | pmid:14713238 |
| Johnson DW et al. | Measurement of plasma pristanic, phytanic and very long chain fatty acids by liquid chromatography-electrospray tandem mass spectrometry for the diagnosis of peroxisomal disorders. | 2003 | J. Chromatogr. B Analyt. Technol. Biomed. Life Sci. | pmid:14630371 |
| Westin MA et al. | Molecular cloning and characterization of two mouse peroxisome proliferator-activated receptor alpha (PPARalpha)-regulated peroxisomal acyl-CoA thioesterases. | 2004 | J. Biol. Chem. | pmid:15007068 |
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| Atshaves BP et al. | Sexually dimorphic metabolism of branched-chain lipids in C57BL/6J mice. | 2004 | J. Lipid Res. | pmid:14993239 |
| Clarke CE et al. | Tremor and deep white matter changes in alpha-methylacyl-CoA racemase deficiency. | 2004 | Neurology | pmid:15249642 |
| Peduto A et al. | Hyperpipecolic acidaemia: a diagnostic tool for peroxisomal disorders. | 2004 | Mol. Genet. Metab. | pmid:15234336 |
| Gloerich J et al. | A phytol-enriched diet induces changes in fatty acid metabolism in mice both via PPARalpha-dependent and -independent pathways. | 2005 | J. Lipid Res. | pmid:15654129 |
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| Huyghe S et al. | Peroxisomal multifunctional protein 2 is essential for lipid homeostasis in Sertoli cells and male fertility in mice. | 2006 | Endocrinology | pmid:16484321 |
| Roe DS et al. | Differentiation of long-chain fatty acid oxidation disorders using alternative precursors and acylcarnitine profiling in fibroblasts. | 2006 | Mol. Genet. Metab. | pmid:16297647 |
| Ferdinandusse S et al. | Mutations in the gene encoding peroxisomal sterol carrier protein X (SCPx) cause leukencephalopathy with dystonia and motor neuropathy. | 2006 | Am. J. Hum. Genet. | pmid:16685654 |