CFTR-dependent chloride efflux in cystic fibrosis mononuclear cells is increased by ivacaftor therapy.
Lorenzo GuerraSusanna D'OriaMaria FaviaStefano CastellaniTeresa SantostasiAngela M PolizziMaria A MariggiòCrescenzio GalloValeria CasavolaPasqualina MontemurroGiuseppina LeonettiAntonio MancaMassimo ConesePublished in: Pediatric pulmonology (2017)
In patients with non-G551D mutations, ivacaftor improved both chloride transport in sweat ducts and chloride efflux in MNC, that is, functions directly imputed to CFTR.