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 are mar...

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Detalhes bibliográficos

Principais autores:	Megumi Honda, G.S. Tint, Akira Honda, Lien B. Nguyen, Thomas S. Chen, Sarah Shefer
Formato:	Artigo
Idioma:	Inglês
Publicado em:	Elsevier 1998-03-01
coleção:	Journal of Lipid Research
Assuntos:	HMG-CoA reductase HMG-CoA synthase ketoconazole LDL receptor plasma sterols
Acesso em linha:	http://www.sciencedirect.com/science/article/pii/S0022227520333022
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