CFTR and cystic fibrosis: Ivacaftor activated CFTR-dependent chloride transport in non-CF epithelia and ameliorated the functional CFTR defect induced by smoke to 185±36% of non-CF control (P<0.05), thereby increasing airway surface liquid (from 7.3±0.6 μm to 10.1±0.4 μm, P<0.005) and mucociliary transport (from 0.27±0.11 μm/s to 2.7±0.28 μm/s, P<0.005).