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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Bibliographic Details
Published in:Mol Cell Pediatr
Main Authors: Esposito, Speranza, Tosco, Antonella, Villella, Valeria R., Raia, Valeria, Kroemer, Guido, Maiuri, Luigi
Format: Artigo
Language:Inglês
Published: Springer Berlin Heidelberg 2016
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Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC4791443/
https://ncbi.nlm.nih.gov/pubmed/26976279
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s40348-016-0040-z
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