Ivacaftor potentiation of multiple CFTR channels with gating mutations.

  title={Ivacaftor potentiation of multiple CFTR channels with gating mutations.},
  author={Haihui Yu and Bill Burton and Chien-Jung Huang and Jennings Worley and Dong Cao and James W. Johnson and Art Urrutia and John Joubran and Sheila D Seepersaud and Katherine Sussky and Beth J. Hoffman and Fredrick Van Goor},
  journal={Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society},
  volume={11 3},
BACKGROUND The investigational CFTR potentiator ivacaftor (VX-770) increased CFTR channel activity and improved lung function in subjects with CF who have the G551D CFTR gating mutation. The aim of this in vitro study was to determine whether ivacaftor potentiates mutant CFTR with gating defects caused by other CFTR gating mutations. METHODS The effects of ivacaftor on CFTR channel open probability and chloride transport were tested in electrophysiological studies using Fischer rat thyroid… CONTINUE READING
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