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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. 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 …
182 results