Trihydroxycholestanoic Acid (Synonyms: Trihydroxycoprostanic Acid) |
Catalog No.GC45945 |
A cholic acid intermediate
Products are for research use only. Not for human use. We do not sell to patients.
Cas No.: 547-98-8
Sample solution is provided at 25 µL, 10mM.
Trihydroxycholestanoic acid is an intermediate in the biosynthesis of cholic acid .1 Elevated plasma levels of trihydroxycholestanoic acid have been found in patients with Zellweger syndrome, a neurological disorder characterized by mutations in PEX genes which result in defects in peroxisome formation.2,3
|1. Keane, M.H., Overmars, H., Wikander, T.M., et al. Bile acid treatment alters hepatic disease and bile acid transport in peroxisome-deficient PEX2 Zellweger mice. Hepatology 45(4), 982-997 (2007).|2. Ferdinandusse, S., Overmars, H., Denis, S., et al. Plasma analysis of di- and trihydroxycholestanoic acid diastereoisomers in peroxisomal α-methylacyl-CoA racemase deficiency. J. Lipid Res. 42(1), 137-141 (2001).|3. Klouwer, F.C.C., Berendse, K., Ferdinandusse, S., et al. Zellweger spectrum disorders: Clinical overview and management approach. Orphanet J. Rare Dis. 10, 151 (2015).
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