In patients with cystic fibrosis (CF), a spectrum of mutations in the CFTR gene leads to deficient CFTR-mediated Cl−/fluid secretion and enhanced ENaC-mediated Na+/fluid absorption resulting in ASL depletion and mucus hyperconcentration, which in turn impairs mucociliary clearance causing mucus obstruction, chronic bacterial infection, inflammation, and progressive structural lung damage2,3,6–8. This evidence concerns the gene CFTR and cystic fibrosis.