CFTR and cystic fibrosis: Although CF is a multi-organ disease, the most severe manifestations are observed in the lung, where the loss of the chloride and bicarbonate secretion mediated by CFTR dehydrates and acidifies the thin layer of fluid covering the airways and impairs the mucociliary clearance and the innate immunity, thus resulting in chronic infection, inflammation, and progressive structural and functional lung damage [9,10,11,12,13,14,15].