Identification of 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 25, 26-pentol in cerebrotendinous xanthomatosis.
An unrecognized pentahydroxy bile alcohol has been isolated from the bile and feces of patients with cerebrotendinous xanthomatosis (CTX). Its structure, 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 25, 26-pentol has been deduced by spectroscopic methods and confirmed by comparison with a synthetic analog.
D001646:Bile / Q000378:metabolism*; D001927:Brain Diseases / Q000378:metabolism*; D002777:Cholestanols / Q000032:analysis*; D002849:Chromatography, Gas; D002855:Chromatography, Thin Layer; D005243:Feces / Q000032:analysis; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D013058:Mass Spectrometry; D010084:Oxidation-Reduction; D013261:Sterols / Q000032:analysis; D014973:Xanthomatosis / Q000378:metabolism*
D002777:Cholestanols; D013261:Sterols; C006836:5 beta-bufol