Differences in hepatic levels of intermediates in bile acid biosynthesis between Cyp27−/− mice and CTX

Differences in hepatic levels of intermediates in bile acid biosynthesis between Cyp27−/− mice and CTX

Cerebrotendinous xanthomatosis (CTX) is a rare, recessively inherited lipid storage disease characterized by a markedly reduced production of chenodeoxycholic acid and an increased formation of 25-hydroxylated bile alcohols and cholestanol. Patients with this disease are known to have mutations in t...

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Bibliographic Details
Main Authors: Akira Honda, Gerald Salen, Yasushi Matsuzaki, Ashok K. Batta, Guorong Xu, Eran Leitersdorf, G. Stephen Tint, Sandra K. Erickson, Naomi Tanaka, Sarah Shefer
Format: Article
Language:English
Published: Elsevier 2001-02-01
Series:Journal of Lipid Research
Subjects:
cholesterol
cholestanol
bile alcohols
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520316916

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