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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Publicat a:Sci Rep
Autors principals: 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
Format: Artigo
Idioma:Inglês
Publicat: Nature Publishing Group UK 2017
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Article
Accés en línia: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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