Despite ground-breaking advances in CF therapies obtained with the discovery of drugs that correct or potentiate the defective CF transmembrane conductance regulator (CFTR) protein, the unrelenting inflammatory response in the airways and persistent, recurrent infections remain the principal cause of progressive lung disease in patients, contributing to the high morbidity and early mortality of CF, with ~800 life losses/year (Cystic Fibrosis Foundation, 2016; Zolin et al., 2017). Here, CFTR is linked to cystic fibrosis.