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
Niemann-Pick Diseases D009542 25 associated lipids
Retinitis Pigmentosa D012174 6 associated lipids
Zellweger Syndrome D015211 39 associated lipids
Dystonia D004421 3 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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Per page 10 20 50 100 | Total 141
Authors Title Published Journal PubMed Link
pmid:
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
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
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
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
pmid:11356170
pmid:11356171
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
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
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
McLean BN et al. A new defect of peroxisomal function involving pristanic acid: a case report. 2002 J. Neurol. Neurosurg. Psychiatr. pmid:11861706
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
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
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Wanders RJ et al. Activation and oxidation of pristanic acid in rat liver: identification of a distinct, clofibrate non-inducible pristanoyl-CoA oxidase. 1992 Prog. Clin. Biol. Res. pmid:1438370
Wanders RJ et al. Molecular analysis of disorders of peroxisomal beta-oxidation. 1992 Prog. Clin. Biol. Res. pmid:1438395
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
Singh I et al. Phytanic acid alpha-oxidation in human cultured skin fibroblasts. 1992 Biochim. Biophys. Acta pmid:1463774
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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
Atshaves BP et al. Sexually dimorphic metabolism of branched-chain lipids in C57BL/6J mice. 2004 J. Lipid Res. pmid:14993239
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
van den Brink DM et al. Identification of fatty aldehyde dehydrogenase in the breakdown of phytol to phytanic acid. 2004 Mol. Genet. Metab. pmid:15110319
Peduto A et al. Hyperpipecolic acidaemia: a diagnostic tool for peroxisomal disorders. 2004 Mol. Genet. Metab. pmid:15234336
Clarke CE et al. Tremor and deep white matter changes in alpha-methylacyl-CoA racemase deficiency. 2004 Neurology pmid:15249642
pmid:15574183
pmid:15644212
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
Atshaves BP et al. Effect of branched-chain fatty acid on lipid dynamics in mice lacking liver fatty acid binding protein gene. 2005 Am. J. Physiol., Cell Physiol. pmid:15692150
Wanders RJ et al. Characteristics and subcellular localization of pristanoyl-CoA synthetase in rat liver. 1992 Biochim. Biophys. Acta pmid:1596515
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
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
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
pmid:16773508
Gloerich J et al. Metabolism of phytol to phytanic acid in the mouse, and the role of PPARalpha in its regulation. 2007 J. Lipid Res. pmid:17015885
Atshaves BP et al. Effect of SCP-x gene ablation on branched-chain fatty acid metabolism. 2007 Am. J. Physiol. Gastrointest. Liver Physiol. pmid:17068117