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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Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Cyhoeddwyd yn:Sci Rep
Prif Awduron: 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
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: Nature Publishing Group UK 2017
Pynciau:
Article
Mynediad Ar-lein:https://ncbi.nlm.nih.gov/pmc/articles/PMC5547155/
https://ncbi.nlm.nih.gov/pubmed/28785019
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41598-017-07504-1
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