Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney

Nephropathic cystinosis is a lysosomal storage disease characterized by proximal tubular cell dysfunction. Here Festa and colleagues show that these lysosomal alterations lead to defective autophagic clearance of mitochondria and increased oxidative stress that, in turn, activates the transcription...

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Bibliographic Details
Main Authors: Beatrice Paola Festa, Zhiyong Chen, Marine Berquez, Huguette Debaix, Natsuko Tokonami, Jenny Ann Prange, Glenn van de Hoek, Cremonesi Alessio, Andrea Raimondi, Nathalie Nevo, Rachel H. Giles, Olivier Devuyst, Alessandro Luciani
Format: Article
Language:English
Published: Nature Portfolio 2018-01-01
Series:Nature Communications
Online Access:https://doi.org/10.1038/s41467-017-02536-7