7-Dehydrocholesterol down-regulates cholesterol biosynthesis in cultured Smith-Lemli-Opitz syndrome skin fibroblasts

7-Dehydrocholesterol down-regulates cholesterol biosynthesis in cultured Smith-Lemli-Opitz syndrome skin fibroblasts

The Smith-Lemli-Opitz syndrome (SLOS) is a common birth defect–mental retardation syndrome caused by a defect in the enzyme that reduces 7-dehydrocholesterol to cholesterol. Because of this block, patients' plasma cholesterol levels are generally low while 7-dehydrocholesterol concentrations ar...

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Bibliographic Details
Main Authors: Megumi Honda, G.S. Tint, Akira Honda, Lien B. Nguyen, Thomas S. Chen, Sarah Shefer
Format: Article
Language:English
Published: Elsevier 1998-03-01
Series:Journal of Lipid Research
Subjects:
HMG-CoA reductase
HMG-CoA synthase
ketoconazole
LDL receptor
plasma sterols
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520333022

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http://www.sciencedirect.com/science/article/pii/S0022227520333022

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