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.

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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

MeSH term MeSH ID Detail
Dystonia D004421 3 associated lipids
Peroxisomal Disorders D018901 5 associated lipids
Retinitis Pigmentosa D012174 6 associated lipids
Brain Damage, Chronic D001925 6 associated lipids
Chondrodysplasia Punctata D002806 8 associated lipids
Brain Diseases, Metabolic, Inborn D020739 10 associated lipids
Abnormalities, Multiple D000015 13 associated lipids
Refsum Disease D012035 19 associated lipids
Cholestasis D002779 23 associated lipids
Niemann-Pick Diseases D009542 25 associated lipids
Per page 10 20 | Total 14

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.

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PubChem Biomolecular Interactions and Pathways

Link to PubChem Biomolecular Interactions and Pathways

What cellular locations are associated with Pristanic acid?

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

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

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

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Authors Title Published Journal PubMed Link
Atshaves BP et al. Sexually dimorphic metabolism of branched-chain lipids in C57BL/6J mice. 2004 J. Lipid Res. pmid:14993239
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
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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
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Zomer AW et al. Phytanic and pristanic acid are naturally occuring ligands. 2003 Adv. Exp. Med. Biol. pmid:14713238
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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
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
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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
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Ferdinandusse S et al. Subcellular localization and physiological role of alpha-methylacyl-CoA racemase. 2000 J. Lipid Res. pmid:11060359
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
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
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
Paton BC et al. Oxidation of pristanic acid in fibroblasts and its application to the diagnosis of peroxisomal beta-oxidation defects. 1996 J. Clin. Invest. pmid:8609223
Kase BF and Björkhem I Studies on the degradation of [U-3H]-phytanic acid and [U-3H]-pristanic acid in cultured fibroblasts from children with peroxisomal disorders. 1996 Scand. J. Clin. Lab. Invest. pmid:8761525
Jakobs BS and Wanders RJ Impaired peroxisomal fatty acid oxidation in human skin fibroblasts with a mitochondrial acylcarnitine/carnitine translocase deficiency. 1996 J. Inherit. Metab. Dis. pmid:8739961
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
Paton BC et al. Biochemical findings in a series of Australian patients with isolated defects in peroxisomal beta-oxidation. 1996 Ann. N. Y. Acad. Sci. pmid:8993614
Jakobs BS and Wanders RJ 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. 1995 Biochem. Biophys. Res. Commun. pmid:7654220
Wanders RJ et al. Measurement of peroxisomal fatty acid beta-oxidation in cultured human skin fibroblasts. 1995 J. Inherit. Metab. Dis. pmid:9053546
Verhoeven NM et al. Pre- and postnatal diagnosis of peroxisomal disorders using stable-isotope dilution gas chromatography--mass spectrometry. 1995 J. Inherit. Metab. Dis. pmid:9053555
Dacremont G et al. Measurement of very long-chain fatty acids, phytanic and pristanic acid in plasma and cultured fibroblasts by gas chromatography. 1995 J. Inherit. Metab. Dis. pmid:9053557
Singh H and Poulos A Substrate specificity of rat liver mitochondrial carnitine palmitoyl transferase I: evidence against alpha-oxidation of phytanic acid in rat liver mitochondria. 1995 FEBS Lett. pmid:7867794