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
|
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
|
Verhoeven NM and Jakobs C |
Human metabolism of phytanic acid and pristanic acid. |
2001 |
Prog. Lipid Res. |
pmid:11591435
|
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
|
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
|
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
|