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...
Main Authors: | , , , |
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Format: | Article |
Language: | English |
Published: |
Elsevier
2014-04-01
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Series: | Neurobiology of Disease |
Subjects: | |
Online Access: | http://www.sciencedirect.com/science/article/pii/S0969996114000059 |