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Lack of Activation of the Unfolded Protein Response in Mouse and Cellular Models of Niemann-Pick Type C Disease

✍ Scribed by Klein, Andres; Mosqueira, Matías; Martínez, Gabriela; Robledo, Fermín; González, Marcela; Caballero, Benjamín; Cancino, Gonzalo I.; Alvarez, Alejandra R.; Hetz, Claudio; Zanlungo, Silvana


Book ID
120333337
Publisher
S. Karger AG
Year
2010
Tongue
English
Weight
260 KB
Volume
8
Category
Article
ISSN
1660-2854

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## Abstract Niemann‐Pick disease type C (NPC) is a progressive neurodegenerative disorder characterized by accumulation of free cholesterol in lysosomes, mainly due to a mutation in the NPC1 gene. The pathophysiological basis of the neural disorders in NPC, however, is not well understood. We found