| Description | 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 |
| Synonyms | Trihydroxycoprostanic Acid |
| molecular weight | 450.66 |
| Molecular formula | C27H46O5 |
| CAS | 547-98-8 |
| Storage | Powder: -20°C for 3 years | In solvent: -80°C for 1 year |
| Solubility | Chloroform: Slightly soluble, Heating is recommended. Ethanol: Slightly soluble DMSO: Slightly soluble |
| References | 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). |