Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators-Reference-Cited by-同舟云学术

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

Published:2017-08-07 Issue:1 Volume:7 Page:
ISSN:2045-2322
Container-title:Scientific Reports
language:en
Short-container-title:Sci Rep

Author:

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^ORCID,Mesbahi Myriam,Girodon-Boulandet Emanuelle,Hinzpeter Alexandre,Edelman Aleksander,Sermet-Gaudelus Isabelle

Publisher

Springer Science and Business Media LLC

Subject

Multidisciplinary

Link

http://www.nature.com/articles/s41598-017-07504-1.pdf

Reference42 articles.

1. O’Sullivan, B. P. & Freedman, S. D. Cystic fibrosis. The Lancet 373, 1891–1904 (2009).

2. Pranke, I. M. & Sermet-Gaudelus, I. Biosynthesis of cystic fibrosis transmembrane conductance regulator. Int. J. Biochem. Cell Biol. 52, 26–38 (2014).

3. Van Goor, F. et al. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc. Natl. Acad. Sci. USA 106, 18825–18830 (2009).

4. Accurso, F. J. et al. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N. Engl. J. Med. 363, 1991–2003 (2010).

5. Accurso, F. J. et al. Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data. J. Cyst. Fibros. 13, 139–147 (2014).

Cited by 130 articles. 订阅此论文施引文献订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献

1. CFTR modulators response of S737F and T465N CFTR variants on patient-derived rectal organoids;Orphanet Journal of Rare Diseases;2024-09-13

2. TLN468 changes the pattern of tRNA used to read through premature termination codons in CFTR;Journal of Cystic Fibrosis;2024-08

3. Comparison of a novel potentiator of CFTR channel activity to ivacaftor in ameliorating mucostasis caused by cigarette smoke in primary human bronchial airway epithelial cells;Respiratory Research;2024-07-10

4. ECFS standards of care on CFTR-related disorders: Identification and care of the disorders;Journal of Cystic Fibrosis;2024-07

5. Prime editing functionally corrects cystic fibrosis-causing CFTR mutations in human organoids and airway epithelial cells;Cell Reports Medicine;2024-05