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
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
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.

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 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
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Harris HM et al. Phytanic acid, pristanic acid, and very-long-chain fatty acid methyl esters measured simultaneously by capillary gas chromatography. 1989 Clin. Chem. pmid:2702761
pmid:27189059
pmid:28566232
pmid:28629946
Singh I et al. Refsum disease: a defect in the alpha-oxidation of phytanic acid in peroxisomes. 1993 J. Lipid Res. pmid:7504046
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
Schmitt K et al. [Zellweger syndrome, neonatal adrenoleukodystrophy or infantile Refsum's disease in a case with generalized peroxisome defect?]. 1993 Wien. Klin. Wochenschr. pmid:7687405
Pahan K et al. Phytanic acid alpha-oxidation in rat liver mitochondria. 1994 Biochim. Biophys. Acta pmid:7803482
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
Wanders RJ et al. Clinical and biochemical characteristics of peroxisomal disorders: an update. 1994 Eur. J. Pediatr. pmid:7957386
ten Brink HJ et al. Heterogeneity in di/trihydroxycholestanoic acidaemia. 1994 Ann. Clin. Biochem. pmid:8060102
Singh H et al. 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. 1994 J. Biol. Chem. pmid:8144536
ten Brink HJ et al. Diagnosis of peroxisomal disorders by analysis of phytanic and pristanic acids in stored blood spots collected at neonatal screening. 1993 Clin. Chem. pmid:8375069
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
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
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
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
Mota CR et al. Rhizomelic chondrodysplasia punctata-like phenotype in a newborn male with normal peroxisomal function. 1997 J Pediatr Orthop B pmid:9039662
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
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
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 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. 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 alpha-oxidation: decarboxylation of 2-hydroxyphytanoyl-CoA to pristanic acid in human liver. 1997 J. Lipid Res. pmid:9374128
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
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. Involvement of microsomal fatty aldehyde dehydrogenase in the alpha-oxidation of phytanic acid. 1998 FEBS Lett. pmid:9662422
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
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
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
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
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