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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...

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Detalles Bibliográficos
Autores principales: Esposito, Speranza, Tosco, Antonella, Villella, Valeria R., Raia, Valeria, Kroemer, Guido, Maiuri, Luigi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2016
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