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Defective autophagy degradation and abnormal tight junction-associated signaling drive epithelial dysfunction in cystinosis

Cystinosis is a lysosomal storage disease due to inactivating mutations in CTNS, the cystinosin transporter that exports cystine out of lysosomes. The lysosomal accumulation of cystine leads to severe dysfunction of the epithelial cells lining the proximal tubule of the kidney, causing defective end...

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Publicado en:Autophagy
Autores principales: Luciani, Alessandro, Festa, Beatrice Paola, Chen, Zhiyong, Devuyst, Olivier
Formato: Artigo
Lenguaje:Inglês
Publicado: Taylor & Francis 2018
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Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC6103718/
https://ncbi.nlm.nih.gov/pubmed/29806776
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1080/15548627.2018.1446625
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