Recovery from liver disease in a Niemann-Pick type C mouse model

Recovery from liver disease in a Niemann-Pick type C mouse model

Loss of function of Niemann-Pick C1 (NPC1) leads to lysosomal free cholesterol storage, resulting in the neurodegenerative disease Niemann-Pick disease type C (NPC). Significant numbers of patients with NPC also suffer from liver disease. Currently, no treatments exist that alter patient outcome, an...

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Bibliographic Details
Main Authors: Naomi L. Sayre, Victoria M. Rimkunas, Mark J. Graham, Rosanne M. Crooke, Laura Liscum
Format: Article
Language:English
Published: Elsevier 2010-08-01
Series:Journal of Lipid Research
Subjects:
lysosomal storage disease
sterol responsive element binding protein-2
liver X receptor
HMG CoA reductase
ATP-binding cassette G5
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520370735

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http://www.sciencedirect.com/science/article/pii/S0022227520370735

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