Ivacaftor modifies cystic fibrosis neutrophil phenotype in subjects with R117H residual function CFTR mutations
Author:
Funder
Vertex Pharmaceuticals
Wellcome Trust
Chief Scientist Office
Publisher
European Respiratory Society (ERS)
Subject
Pulmonary and Respiratory Medicine
Reference15 articles.
1. Origins of Cystic Fibrosis Lung Disease
2. A neutrophil intrinsic impairment affecting Rab27a and degranulation in cystic fibrosis is corrected by CFTR potentiator therapy
3. Delayed neutrophil apoptosis enhances NET formation in cystic fibrosis
4. On the mechanism of gating defects caused by the R117H mutation in cystic fibrosis transmembrane conductance regulator
5. Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial;Moss;Lancet Respir Med,2015
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