Acknowledgments
We thank Hermann Tenor (TOPADUR Pharma AG, Schlieren, Switzerland) for fruitful discussion. This study was initiated at the Fondazione Mario Negri Sud where, most of the in vitro experiments were performed until the closure of the Institution, because of failure, in May 2015. Thereafter, the project was continued and concluded at the Department of Medical, Oral and Biotechnological Sciences, G. D’Annunzio University of Chieti-Pescara; Center for Advanced Studies and Technology (CAST), G. D’Annunzio University of Chieti-Pescara. This work was supported by the Italian Cystic Fibrosis Research Foundation (project FFC#21/2011, sponsor Philip Watch - Morellato & Sector Group and FFC#16/2013 sponsor: LIFC con Associazioni Regionali per la Campagna Nazionale FFC, LIFC Associazione Emiliana Onlus, Delegazione FFC di Lecce) to V.E.; by Fondazione Negri Sud Onlus (V.E.); MIUR D.M. 44/08 (V.E.); and by the Italian Ministry of Health (L548/93 to V.E. and M.R.). R.P., A.P, R.P. received fellowships by the Italian Cystic Fibrosis Research Foundation. A.R., S.R., I.D.F. and A.B. are supported by the Italian Cystic Fibrosis Research Foundation (CFaCore).