Ivacaftor restores CFTR-dependent sweat gland fluid secretion in cystic fibrosis subjects with S945L alleles
Author:
Funder
CFFT
Publisher
Elsevier BV
Subject
Pulmonary and Respiratory Medicine,Pediatrics, Perinatology, and Child Health
Reference19 articles.
1. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770;Van Goor;Proc Natl Acad Sci U S A,2009
2. Ivacaftor potentiation of multiple CFTR channels with gating mutations;Yu;J Cyst Fibros,2012
3. Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function;Van Goor;J Cyst Fibros,2014
4. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation;Accurso;N Engl J Med,2010
5. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation;Ramsey;N Engl J Med,2011
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1. S945L-CFTR molecular dynamics, functional characterization and tezacaftor/ivacaftor efficacy in vivo and in vitro in matched pediatric patient-derived cell models;Frontiers in Pediatrics;2022-11-16
2. Expanding CFTR Modulator Testing to Carriers of CFTR Variants;Annals of the American Thoracic Society;2021-11
3. Ciliary Localization of the Intraflagellar Transport Protein IFT88 Is Disrupted in Cystic Fibrosis;American Journal of Respiratory Cell and Molecular Biology;2020-01
4. Salivary Thiocyanate as a Biomarker of Cystic Fibrosis Transmembrane Regulator Function;Analytical Chemistry;2019-05-22
5. Cystic fibrosis year in review 2018, part 1;Pediatric Pulmonology;2019-05-20
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