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 …