Cholesterol Accumulation Is Associated with Lysosomal Dysfunction and Autophagic Stress in Npc1(−/−) Mouse Brain

Niemann-Pick type C (NPC) disease is an autosomal recessive disorder caused by mutations of NPC1 and NPC2 genes. Progressive neurodegeneration that accompanies NPC is fatal, but the underlying mechanisms are still poorly understood. In the present study, we characterized the association of autophagi...

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Detalhes bibliográficos
Main Authors: Liao, Guanghong, Yao, Yueqin, Liu, Jihua, Yu, Zhang, Cheung, Simon, Xie, Ang, Liang, Xiaoli, Bi, Xiaoning
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Investigative Pathology 2007
Assuntos:
Regular Articles
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC1959498/
https://ncbi.nlm.nih.gov/pubmed/17631520
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.2353/ajpath.2007.070052
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