Identification of pentahydroxy bile alcohols in cerebrotendinous xanthomatosis: characterization of 5beta-cholestane-3alpha, 7alpha, 12alpha, 24xi, 25-pentol and 5beta-cholestane-3alpha, 7alpha, 12alpha, 23xi, 25-pentol.

Abstract

This paper describes studies dealing with the nature of the C27 pentahydroxy bile alcohols present in the bile and feces of two patients with cerebrotendinous xanthomatosis (CTX). The presence of a bile alcohol having the structure 5beta-cholestane-3alpha,7alpha,12alpha,24alpha,25-pentol was confirmed by separation of the two 24-hydroxy epimers of 5beta-cholestane-3alpha,7alpha,12alpha,24,25-pentol and characterization of the dpimers by gas-liquid chromatography and infrared and mass spectrometry. Tentative assignment of the 24alpha and 24beta configuration was made on the basis of molecular rotation differences. A second major bile alcohol excreted by the CTX subjects was 5beta-cholestane-3alpha,7alpha,12alpha,23xi,25-pentol. Its structure was determined by infrared spectrometry, proton magnetic resonance spectrometry, and mass spectrometry because a reference compound was not available.

• Authors: S Shefer, B Dayal, G S Tint, G Salen, E H Mosbach
• Journal: Journal of lipid research (J Lipid Res) Vol. 16 Issue 4 Pg. 280-6 (Jul 1975) ISSN: 0022-2275 [Print] United States
• PMID: 1141769 (Publication Type: Journal Article, Research Support, U.S. Gov't, Non-P.H.S., Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Hydroxysteroids
• Sterols

Topics

• Bile (metabolism)
• Chromatography, Gas
• Humans
• Hydroxysteroids (metabolism)
• Magnetic Resonance Spectroscopy
• Mass Spectrometry
• Optical Rotation
• Spectrophotometry, Infrared
• Stereoisomerism
• Sterols (metabolism)
• Xanthomatosis (metabolism)