Identification of 5 beta-cholestane-3 alpha, 7 alpha, 12 alpha, 25, 26-pentol in cerebrotendinous xanthomatosis

Steroids. 1983 Oct;42(4):441-8. doi: 10.1016/0039-128x(83)90142-3.

Abstract

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.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Bile / metabolism*
  • Brain Diseases / metabolism*
  • Cholestanols / analysis*
  • Chromatography, Gas
  • Chromatography, Thin Layer
  • Feces / analysis
  • Humans
  • Magnetic Resonance Spectroscopy
  • Mass Spectrometry
  • Oxidation-Reduction
  • Sterols / analysis
  • Xanthomatosis / metabolism*

Substances

  • Cholestanols
  • Sterols
  • 5 beta-bufol