Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) that prevent its proper folding and trafficking to the apical membrane of epithelial
cells. Absence of cAMP-mediated Cl− secretion in CF airways causes poorly hydrated
airway surfaces in CF patients, and this condition is exacerbated by excessive Na+
absorption. The mechanistic link between missing CFTR and increased Na+ absorption in
airway epithelia has remained elusive, although substantial evidence implicates …