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Bile acid synthesis in the Smith-Lemli-Opitz syndrome: effects of dehydrocholesterols on cholesterol 7α-hydroxylase and 27-hydroxylase activities in rat liver

The Smith-Lemli-Opitz syndrome (SLOS) is a congenital birth defect syndrome caused by a deficiency of 3β-hydroxysterol Δ7-reductase, the final enzyme in the cholesterol biosynthetic pathway. The patients have reduced plasma and tissue cholesterol concentrations with the accumulation of 7-dehydrochol...

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Principais autores: Akira Honda, Gerald Salen, Sarah Shefer, Ashok K. Batta, Megumi Honda, Guorong Xu, G. Stephen Tint, Yasushi Matsuzaki, Junichi Shoda, Naomi Tanaka
Formato: Artigo
Idioma:Inglês
Publicado em: Elsevier 1999-08-01
coleção:Journal of Lipid Research
Assuntos:
7-dehydrocholesterol
8-dehydrocholesterol
Acesso em linha:http://www.sciencedirect.com/science/article/pii/S0022227520333964
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