Ivacaftor potentiation of multiple CFTR channels with gating mutations by Yu Haihui, Burton Bill, Huang Chien-Jung, Worley Jennings, Cao Dong, Johnson James P, Urrutia Art, Joubran John, Seepersaud Sheila, Sussky Katherine, Hoffman Beth J, Van Goor Fredrick in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society (2012). PubMed

Abstract

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.

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