Evaluation of Two Liver Treatment Strategies in a Mouse Model of Niemann–Pick-Disease Type C1

Evaluation of Two Liver Treatment Strategies in a Mouse Model of Niemann–Pick-Disease Type C1

Niemann–Pick-disease type C1 (NPC1) is an autosomal-recessive cholesterol-storage disorder. Besides other symptoms, NPC1 patients develop liver dysfunction and hepatosplenomegaly. The mechanisms of hepatomegaly and alterations of lipid metabolism-related genes in NPC1 disease are still poorly unders...

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Bibliographic Details
Main Authors: Lynn Ebner, Anne Gläser, Anja Bräuer, Martin Witt, Andreas Wree, Arndt Rolfs, Marcus Frank, Brigitte Vollmar, Angela Kuhla
Format: Article
Language:English
Published: MDPI AG 2018-03-01
Series:International Journal of Molecular Sciences
Subjects:
NPC1 mutant
liver dysfunction
miglustat
allopregnanolone
cyclodextrin
Online Access:http://www.mdpi.com/1422-0067/19/4/972

Internet

http://www.mdpi.com/1422-0067/19/4/972

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