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Plasma analysis of di- and trihydroxycholestanoic acid diastereoisomers in peroxisomal alpha-methylacyl-CoA racemase deficiency.
Ferdinandusse S, Overmars H, Denis S, Waterham HR, Wanders RJ, Vreken P. Ferdinandusse S, et al. Among authors: denis s. J Lipid Res. 2001 Jan;42(1):137-41. J Lipid Res. 2001. PMID: 11160375 Free article.
Patients with this disorder show elevated plasma levels of pristanic acid and the bile acid intermediates di- and trihydroxycholestanoic acid (DHCA and THCA), which are all substrates for the peroxisomal beta-oxidation system. alpha-Methylacyl-CoA racemase plays an important role …
Patients with this disorder show elevated plasma levels of pristanic acid and the bile acid intermediates di- and trihydroxycholestanoic aci …
Molecular analysis of disorders of peroxisomal beta-oxidation.
Wanders RJ, van Roermund CW, Denis S, Schutgens RB, Ijlst L, Tager JM. Wanders RJ, et al. Among authors: denis s. Prog Clin Biol Res. 1992;375:507-19. Prog Clin Biol Res. 1992. PMID: 1438395 Review. No abstract available.
Major hyperpipecolataemia in a normal adult.
Vallat C, Denis S, Bellet H, Jakobs C, Wanders RJ, Mion H. Vallat C, et al. Among authors: denis s. J Inherit Metab Dis. 1996;19(5):624-6. doi: 10.1007/BF01799837. J Inherit Metab Dis. 1996. PMID: 8892018
Phytanoyl-CoA hydroxylase is present in human liver, located in peroxisomes, and deficient in Zellweger syndrome: direct, unequivocal evidence for the new, revised pathway of phytanic acid alpha-oxidation in humans.
Jansen GA, Mihalik SJ, Watkins PA, Moser HW, Jakobs C, Denis S, Wanders RJ. Jansen GA, et al. Among authors: denis s. Biochem Biophys Res Commun. 1996 Dec 4;229(1):205-10. doi: 10.1006/bbrc.1996.1781. Biochem Biophys Res Commun. 1996. PMID: 8954107 Free article.
Metabolic aspects of peroxisomal disorders.
Wanders RJ, Jansen G, van Roermund CW, Denis S, Schutgens RB, Jakobs BS. Wanders RJ, et al. Among authors: denis s. Ann N Y Acad Sci. 1996 Dec 27;804:450-60. doi: 10.1111/j.1749-6632.1996.tb18636.x. Ann N Y Acad Sci. 1996. PMID: 8993564
216 results