HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Batta, A. K. Articles by Shefer, S. Search for Related Content PubMed PubMed Citation Articles by Batta, A. K. Articles by Shefer, S. Social Bookmarking                      What's this?

Journal of Lipid Research, Vol 36, 2413-2418, Copyright © 1995 by Lipid Research, Inc.

ARTICLES

Identification of 19-nor-5,7,9(10)-cholestatrien-3 beta-ol in patients with Smith-Lemli-Opitz syndrome

AK Batta, G Salen, GS Tint and S Shefer
Department of Medicine, University of Medicine and Dentistry-New Jersey Medical School, Newark, USA.

We have identified the third unknown sterol in the plasma and tissues of Smith-Lemli-Opitz homozygotes as 19-nor-5,7,9(10)-cholestatrien-3 beta-ol. The structure was established from capillary gas-liquid chromatography retention index and characteristic fragmentation pattern by mass spectrometry that were identical to a synthetic reference standard. Evidence is presented that 19-nor-5,7,9(10)-cholestatrien-3 beta-ol is not an artifact formed during the chemical isolation of the relatively unstable 7-dehydrocholesterol. It is possible that 19-nor- 5,7,9(10)-cholestatrien-3 beta-ol may contribute to the clinical abnormalities in patients with Smith-Lemli-Opitz syndrome.
CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

				B. Lindenthal, T. A. Aldaghlas, J. K. Kelleher, S. M. Henkel, R. Tolba, G. Haidl, and K. von Bergmann Neutral sterols of rat epididymis: high concentrations of dehydrocholesterols in rat caput epididymidis J. Lipid Res., July 1, 2001; 42(7): 1089 - 1095. [Abstract] [Full Text] [PDF]

				B. Ruan, W. K. Wilson, J. Pang, N. Gerst, F. D. Pinkerton, J. Tsai, R. I. Kelley, F. G. Whitby, D. M. Milewicz, J. Garbern, et al. Sterols in blood of normal and Smith-Lemli-Opitz subjects J. Lipid Res., May 1, 2001; 42(5): 799 - 812. [Abstract] [Full Text]

				R. D. Steiner, L. M. Linck, D. P. Flavell, D. S. Lin, and W. E. Connor Sterol balance in the Smith-Lemli-Opitz syndrome: reduction in whole body cholesterol synthesis and normal bile acid production J. Lipid Res., September 1, 2000; 41(9): 1437 - 1447. [Abstract] [Full Text]

				W. Gaoua, F. Chevy, C. Roux, and C. Wolf Oxidized derivatives of 7-dehydrocholesterol induce growth retardation in cultured rat embryos: a model for antenatal growth retardation in the Smith-Lemli-Opitz syndrome J. Lipid Res., March 1, 1999; 40(3): 456 - 463. [Abstract] [Full Text]

				P. T Clayton Disorders of cholesterol biosynthesis Arch. Dis. Child., February 1, 1998; 78(2): 185 - 189. [Full Text]