Identification of 8-dehydrocholesterol (cholesta-5,8-dien-3 beta-ol) in patients with Smith-Lemli-Opitz syndrome.

Abstract

Cholesta-5,8-dien-3 beta-ol (8-dehydrocholesterol) and cholesta-5,7-dien-3 beta-ol (7-dehydrocholesterol) were isolated from the fecal neutral sterol fraction from homozygotes with Smith-Lemli-Opitz syndrome. The structures of the sterols were conclusively established from their mass spectra and 1H and 13C nuclear magnetic resonance spectra. It is probable that 8-dehydrocholesterol arises from 7-dehydrocholesterol and is not a direct precursor of cholesterol.

• Authors: A K Batta, G S Tint, S Shefer, D Abuelo, G Salen
• Journal: Journal of lipid research (J Lipid Res) Vol. 36 Issue 4 Pg. 705-13 (Apr 1995) ISSN: 0022-2275 [Print] United States
• PMID: 7616117 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, Non-P.H.S., Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Dehydrocholesterols
• 7-dehydrocholesterol

Topics

• Abnormalities, Multiple (metabolism)
• Dehydrocholesterols (chemistry, isolation & purification)
• Feces (chemistry)
• Humans
• Lipid Metabolism, Inborn Errors (metabolism)
• Magnetic Resonance Spectroscopy
• Molecular Structure
• Syndrome