The exact pathophysiology linking cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction with CF pulmonary disease remains unclear, although the popular airway surface liquid (ASL) volume theory [1,2] predicts that dehydrated, thickened respiratory secretions are integral to mucociliary stasis and the subsequent development of CF airway pathology. Here, CFTR is linked to cystic fibrosis.