Disrupted coordinate regulation of farnesoid X receptor target genes in a patient with cerebrotendinous xanthomatosis

Disrupted coordinate regulation of farnesoid X receptor target genes in a patient with cerebrotendinous xanthomatosis

Cerebrotendinous xanthomatosis (CTX), sterol 27-hydroxylase (CYP27A1) deficiency, is associated with markedly reduced chenodeoxycholic acid (CDCA), the most powerful activating ligand for farnesoid X receptor (FXR). We investigated the effects of reduced CDCA on FXR target genes in humans. Liver spe...

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Bibliographic Details
Main Authors: Akira Honda, Gerald Salen, Yasushi Matsuzaki, Ashok K. Batta, Guorong Xu, Takeshi Hirayama, G. Stephen Tint, Mikio Doy, Sarah Shefer
Format: Article
Language:English
Published: Elsevier 2005-02-01
Series:Journal of Lipid Research
Subjects:
bile acids
bile alcohols
sterol 27-hydroxylase
cholesterol 7α-hydroxylase
Na+/taurocholate-cotransporting polypeptide
bile salt export pump
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520340621

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http://www.sciencedirect.com/science/article/pii/S0022227520340621

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