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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Autores principales:	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
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2018
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/72c5aea539634421966302e0bad09721
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spelling	oai:doaj.org-article:72c5aea539634421966302e0bad097212021-12-02T15:33:41ZImpaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney10.1038/s41467-017-02536-72041-1723https://doaj.org/article/72c5aea539634421966302e0bad097212018-01-01T00:00:00Zhttps://doi.org/10.1038/s41467-017-02536-7https://doaj.org/toc/2041-1723Nephropathic 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 factor ZONAB leading to impaired cell differentiation.Beatrice Paola FestaZhiyong ChenMarine BerquezHuguette DebaixNatsuko TokonamiJenny Ann PrangeGlenn van de HoekCremonesi AlessioAndrea RaimondiNathalie NevoRachel H. GilesOlivier DevuystAlessandro LucianiNature PortfolioarticleScienceQENNature Communications, Vol 9, Iss 1, Pp 1-17 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q 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 Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney
description	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 factor ZONAB leading to impaired cell differentiation.
format	article
author	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
author_facet	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
author_sort	Beatrice Paola Festa
title	Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney
title_short	Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney
title_full	Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney
title_fullStr	Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney
title_full_unstemmed	Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney
title_sort	impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney
publisher	Nature Portfolio
publishDate	2018
url	https://doaj.org/article/72c5aea539634421966302e0bad09721
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Impaired autophagy bridges lysosomal storage disease and epithelial dysfunction in the kidney

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