The autophagic defect in Niemann–Pick disease type C neurons differs from somatic cells and reduces neuronal viability

Niemann–Pick disease type C (NPC) is a fatal, progressive neurovisceral disorder. Several studies report that the autophagic flux is disturbed in NPC1-deficient (NPC1−/−) cells. Since it has been suggested that the autophagic defect may contribute to the neurodegeneration, we used cell cultures of N...

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Bibliographic Details
Main Authors: Volker Meske, Jennifer Erz, Timm Priesnitz, Thomas-Georg Ohm
Format: Article
Language:English
Published: Elsevier 2014-04-01
Series:Neurobiology of Disease
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996114000059