Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.

  title={Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.},
  author={Fredrick Van Goor and Sabine Hadida and Peter D. J. Grootenhuis and Bill Burton and Dong Cao and Tim Neuberger and Amanda Turnbull and Ashvani K Singh and John Joubran and Anna Hazlewood and Jinglan Zhou and Jason S. McCartney and Vijayalaksmi Arumugam and Caroline Decker and Jennifer Marie J Yang and Chris R Young and Eric R. Olson and Jeffery J Wine and Raymond A Frizzell and Melissa A Ashlock and Paul A. Negulescu},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  volume={106 44},
Cystic fibrosis (CF) is a fatal genetic disease caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR), a protein kinase A (PKA)-activated epithelial anion channel involved in salt and fluid transport in multiple organs, including the lung. Most CF mutations either reduce the number of CFTR channels at the cell surface (e.g., synthesis or processing mutations) or impair channel function (e.g., gating or conductance mutations) or both. There are currently no… CONTINUE READING
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