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

doi:10.1038/s41598-017-07504-1

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Article

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

Scientific reports - 7 Aug 2017

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

Abstract excerpt

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 study was to determine a biomarker predictive of the clinical response. CFTR function was assessed in vivo via nasal...

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PMID: 28785019
DOI: 10.1038/s41598-017-07504-1
PMCID: PMC5547155

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CFTR

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