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A more recent version of this article appeared on June 1, 2005

Papers In Press, published online ahead of print April 1, 2005
J. Lipid Res., doi:10.1194/jlr.M400462-JLR200
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Submitted on November 17, 2004
Revised on March 17, 2005
Accepted on March 21, 2005

Changes in gene expression associated with loss of function of the NSDHL sterol dehydrogenase in mouse embryonic fibroblasts

David Cunningham, Daniel Swartzlander, Sandya Liyanarachchi, Ramana V. Davuluri, and Gail E. Herman

Department of Human and Molecular Genetics, Columbus Children's Research Institute, Columbus, OH 43205

Corresponding Author: hermang{at}pediatrics.ohio-state.edu

Seven human disorders of post-squalene cholesterol biosynthesis have been described. One of these, CHILD syndrome, results from mutations in the X-linked gene NSDHL encoding a sterol dehydrogenase. A series of mutant alleles of the murine Nsdhl gene are carried by bare patches (Bpa) mice, with Bpa1H representing a null allele. Heterozygous Bpa1H females display skin and skeletal abnormalities in a distribution reflecting random X-inactivation, while hemizygous male embryos die before E10.5. To investigate the molecular basis of defects associated with perturbations in cholesterol biosynthesis, microarray analysis was performed comparing gene expression in embryonic fibroblasts expressing the Bpa1H allele versus wild type cells. Labeled cDNAs from cells grown in normal or lipid-depleted sera (LDS) were hybridized to microarrays containing 22,000 mouse genes. Among 44 genes that showed higher expression in the Bpa1H vs wild type cells grown in LDS, 11 function in cholesterol biosynthesis, 7 are involved in fatty acid synthesis, 3 (Srebp2, Insig1, Orf11) encode sterol regulatory proteins, and 2 (Ldlr, StarD4) are lipid transporters. Of the 21 remaining genes, 16 are known genes, some of which have been previously implicated in cholesterol homeostasis or lipid-mediated signaling, and 5 are uncharacterized cDNA clones.


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