Lipid-mediated motor-adaptor sequestration impairs axonal lysosome delivery leading to autophagic stress and dystrophy in Niemann-Pick type C

Niemann-Pick disease type C (NPC) is a neurodegenerative lysosomal storage disorder characterized by lipid accumulation in endolysosomes. An early pathologic hallmark is axonal dystrophy occurring at presymptomatic stages in NPC mice. However, the mechanisms underlying this pathologic change remain...

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Publicado en:Dev Cell
Main Authors: Roney, Joseph C, Li, Sunan, Farfel-Becker, Tamar, Huang, Ning, Sun, Tao, Xie, Yuxiang, Cheng, Xiu-Tang, Lin, Mei-Yao, Platt, Frances M, Sheng, Zu-Hang
Formato: Artigo
Idioma:Inglês
Publicado: 2021
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Article
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC8137671/
https://ncbi.nlm.nih.gov/pubmed/33878344
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.devcel.2021.03.032
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