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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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Bibliografiska uppgifter
I publikationen:Autophagy
Huvudupphovsmän: Luciani, Alessandro, Festa, Beatrice Paola, Chen, Zhiyong, Devuyst, Olivier
Materialtyp: Artigo
Språk:Inglês
Publicerad: Taylor & Francis 2018
Ämnen:
Länkar: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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