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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I publikationen:Dev Cell
Huvudupphovsmän: 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
Materialtyp: Artigo
Språk:Inglês
Publicerad: 2021
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Article
Länkar: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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