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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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Detalhes bibliográficos
Publicado no:Sci Rep
Main Authors: 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: Artigo
Idioma:Inglês
Publicado em: Nature Publishing Group UK 2017
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Acesso em linha: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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