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
Chow CW et al. Autopsy findings in two siblings with infantile Refsum disease. 1992 Acta Neuropathol. pmid:1373019
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
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. 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
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
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
Kase BF et al. Separation of phytanic and pristanic acid by high-pressure liquid chromatography: application of the method. 1991 Anal. Biochem. pmid:1888042
ten Brink HJ et al. Heterogeneity in di/trihydroxycholestanoic acidaemia. 1994 Ann. Clin. Biochem. pmid:8060102
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
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
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
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
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
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
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
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
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
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
Singh I et al. Phytanic acid alpha-oxidation in human cultured skin fibroblasts. 1992 Biochim. Biophys. Acta pmid:1463774