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Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis
Cystic fibrosis (CF) is a lethal monogenic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that entails the (diagnostic) increase in sweat electrolyte concentrations, progressive lung disease with chronic inflammation and recurrent bacterial infecti...
Autores principales: | Esposito, Speranza, Tosco, Antonella, Villella, Valeria R., Raia, Valeria, Kroemer, Guido, Maiuri, Luigi |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4791443/ https://www.ncbi.nlm.nih.gov/pubmed/26976279 http://dx.doi.org/10.1186/s40348-016-0040-z |
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