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The magnitude of ivacaftor effects on fluid secretion via R117H-CFTR channels: Human in vivo measurements

  • Published:2017-04-18 Issue:4 Volume:12 Page:e0175486
  • ISSN:1932-6203
  • Container-title:PLOS ONE
  • language:en
  • Short-container-title:PLoS ONE

Author:

Char Jessica E.,Dunn Colleen,Davies Zoe,Milla Carlos,Moss Richard B.,Wine Jeffrey J.

Funder

Cystic Fibrosis Foundation Therapeutics

Publisher

Public Library of Science (PLoS)

Subject

Multidisciplinary

Reference47 articles.

1. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770;F Van Goor;Proceedings of the National Academy of Sciences of the United States of America,2009

2. Ivacaftor potentiation of multiple CFTR channels with gating mutations;H Yu;Journal of cystic fibrosis: official journal of the European Cystic Fibrosis Society,2012

3. Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation;FJ Accurso;The New England journal of medicine,2010

4. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation;BW Ramsey;The New England journal of medicine,2011

5. Normalization of sweat chloride concentration and clinical improvement with ivacaftor in a patient with cystic fibrosis with mutation S549N;ME McGarry;Chest,2013

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