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pristanic acid

Known as: 2,6,10, 14-tetramethylpentadecanoic acid 
 
National Institutes of Health

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1 relation

Broader (1)

Fatty Acids

Papers overview

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Highly Cited
2003
Highly Cited
2003
Liver disease caused by failure to racemize trihydroxycholestanoic acid: gene mutation and effect of bile acid therapy.
BACKGROUND & AIMS Inborn errors of bile acid metabolism may present as neonatal cholestasis and fat-soluble vitamin malabsorption… Expand
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Review
2001
Review
2001
Human metabolism of phytanic acid and pristanic acid.
Phytanic acid is a methyl-branched fatty acid present in the human diet. Due to its structure, degradation by beta-oxidation is… Expand
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Highly Cited
2000
Highly Cited
2000
Pristanic acid and phytanic acid: naturally occurring ligands for the nuclear receptor peroxisome proliferator-activated receptor alpha.
Phytanic acid and pristanic acid are branched-chain fatty acids, present at micromolar concentrations in the plasma of healthy… Expand
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Highly Cited
2000
Highly Cited
2000
Subcellular localization and physiological role of alpha-methylacyl-CoA racemase.
alpha-Methylacyl-CoA racemase plays an important role in the beta-oxidation of branched-chain fatty acids and fatty acid… Expand
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Highly Cited
1999
Highly Cited
1999
Human very-long-chain acyl-CoA synthetase: cloning, topography, and relevance to branched-chain fatty acid metabolism.
Very-long-chain acyl-CoA synthetases (VLCS) activate very-long-chain fatty acids (VLCFA) containing 22 or more carbons to their… Expand
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Highly Cited
1998
Highly Cited
1998
Phytanic acid and pristanic acid are oxidized by sequential peroxisomal and mitochondrial reactions in cultured fibroblasts.
The relationship between peroxisomal and mitochondrial oxidation of the methyl branched fatty acids, phytanic acid and pristanic… Expand
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Highly Cited
1998
Highly Cited
1998
Rapid stable isotope dilution analysis of very-long-chain fatty acids, pristanic acid and phytanic acid using gas chromatography-electron impact mass spectrometry.
A common feature of most peroxisomal disorders is the accumulation of very-long-chain fatty acids (VLCFAs) and/or pristanic and… Expand
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Highly Cited
1997
Highly Cited
1997
Evidence that multifunctional protein 2, and not multifunctional protein 1, is involved in the peroxisomal beta-oxidation of pristanic acid.
The second (enoyl-CoA hydratase) and third (3-hydroxyacyl-CoA dehydrogenase) steps of peroxisomal beta-oxidation are catalysed by… Expand
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Highly Cited
1993
Highly Cited
1993
The CoA esters of 2-methyl-branched chain fatty acids and of the bile acid intermediates di- and trihydroxycoprostanic acids are oxidized by one single peroxisomal branched chain acyl-CoA oxidase in…
Rat liver peroxisomes contain three acyl-CoA oxidases: palmitoyl-CoA oxidase, which oxidizes the CoA esters of straight chain… Expand
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1969
1969
Refsum's disease: defective oxidation of phytanic acid in tissue cultures derived from homozygotes and heterozygotes.
Abstract A defect in phytanic acid oxidation was demonstrated in cultured fibroblasts derived from skin biopsies of 11 patients… Expand
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