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Originally published In Press as doi:10.1194/jlr.M300232-JLR200 on November 1, 2003

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Journal of Lipid Research, Vol. 45, 347-355, February 2004
Copyright © 2004 by American Society for Biochemistry and Molecular Biology

Formation of 7-dehydrocholesterol-containing membrane rafts in vitro and in vivo, with relevance to the Smith-Lemli-Opitz syndrome

R. Kennedy Keller*, Thomas P. Arnold{dagger} and Steven J. Fliesler1,§

* Department of Biochemistry and Molecular Biology, University of South Florida College of Medicine, Tampa, FL
{dagger} Department of Biology, Valencia Community College, Orlando, FL
§ Departments of Ophthalmology (St. Louis University Eye Institute) and Pharmacological and Physiological Science, St. Louis University School of Medicine, St. Louis, MO

1 To whom correspondence should be addressed. e-mail: fliesler{at}slu.edu

Smith-Lemli-Opitz syndrome (SLOS) is a recessive disease typified by 7-dehydrocholesterol (7DHC) accumulation and depletion of cholesterol. Because cholesterol is a primary component of detergent-resistant membrane domains ("rafts"), we examined the compatibility of 7DHC with raft formation. Liposomes containing bovine brain phosphatidylcholine, sphingomyelin, cerebrosides, and either cholesterol, 7DHC, or coprostanol (the latter being incompatible with raft formation) were prepared. 7DHC was indistinguishable from cholesterol in its ability to become incorporated into membrane rafts, as judged by physical and chemical criteria, whereas coprostanol did not form rafts. The in vivo compatibility of 7DHC with raft formation was evaluated in brains of rats treated with trans-1,4-bis(2-dichlorobenzylamino-ethyl)cyclohexane dihydrochloride (AY9944), which mimics the SLOS biochemical defect. 7DHC/cholesterol ratios in rafts and whole brains from AY9944-treated rats were similar, indicating comparable efficiency of 7DHC and cholesterol incorporation into brain rafts. In contrast, dolichol (a nonsterol isoprenoid incompatible with raft formation) was greatly depleted in brain rafts relative to whole brain.

Although brain raft fractions prepared from AY9944-treated and control rats yielded similar sterol-protein ratios, their gel electrophoresis profiles exhibited multiple differences, suggesting that altered raft sterol composition perturbs raft protein content. These results are discussed in the context of the SLOS phenotype, particularly with regard to the associated central nervous system defects.

Abbreviations: AY9944, trans-1,4-bis(2-dichlorobenzylaminoethyl) cyclohexane dihydrochloride; CB, galactocerebroside; 7DHC, 7-dehydrocholesterol; DOL, dolichol; HRP, horseradish peroxidase; MBS, MES-buffered saline; PC, phosphatidylcholine; SCAP, SREBP cleavage-activating protein; Shh, Sonic hedgehog; SLOS, Smith-Lemli-Opitz syndrome; SM, sphingomyelin; SREBP, sterol regulatory element binding protein; SSD, sterol-sensing domain

Supplementary key words lipid domains • proteomics • brain • rat


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