Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators

Clinical studies with modulators of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) protein have demonstrated that functional restoration of the mutated CFTR can lead to substantial clinical benefit. However, studies have shown highly variable patient responses. The objective of this...

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Detalles Bibliográficos
Autores principales: Pranke, Iwona M., Hatton, Aurélie, Simonin, Juliette, Jais, Jean Philippe, Le Pimpec-Barthes, Françoise, Carsin, Ania, Bonnette, Pierre, Fayon, Michael, Stremler-Le Bel, Nathalie, Grenet, Dominique, Thumerel, Matthieu, Mazenq, Julie, Urbach, Valerie, Mesbahi, Myriam, Girodon-Boulandet, Emanuelle, Hinzpeter, Alexandre, Edelman, Aleksander, Sermet-Gaudelus, Isabelle
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2017
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5547155/
https://www.ncbi.nlm.nih.gov/pubmed/28785019
http://dx.doi.org/10.1038/s41598-017-07504-1

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