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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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| I publikationen: | Autophagy |
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| Huvudupphovsmän: | , , , |
| Materialtyp: | Artigo |
| Språk: | Inglês |
| Publicerad: |
Taylor & Francis
2018
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| 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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