Lysosomal unesterified cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease

Lysosomal unesterified cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease

Niemann-Pick type C (NPC) disease is a multisystem disorder resulting from mutations in the NPC1 gene that encodes a protein involved in intracellular cholesterol trafficking. Significant liver dysfunction is frequently seen in patients with this disease. The current studies used npc1 mutant mice to...

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Bibliographic Details
Main Authors: Eduardo P. Beltroy, Benny Liu, John M. Dietschy, Stephen D. Turley
Format: Article
Language:English
Published: Elsevier 2007-04-01
Series:Journal of Lipid Research
Subjects:
hepatic dysfunction
chylomicron cholesterol
low density lipoprotein receptor
biliary bile acid
hepatomegaly
small intestine
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520434406

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