In CF, the reduced chloride and bicarbonate secretion caused by loss of function of the cystic fibrosis transmembrane conductance regulator (CFTR) channel disrupts the normal airway homeostasis and leads to a series of pathological alterations including ASL dehydration and acidification, sticky mucus accumulation, mucociliary transport impairment, bacteria colonization, chronic inflammation, and lung tissue damage (Figure 1) [16]. The gene discussed is CFTR; the disease is cystic fibrosis.