Key points• Lung hydration and mucus clearance rates are set by a balance between CFTR‐
mediated Cl− secretion and ENaC‐led Na+ absorption. In CF airways, CFTR is diminished …

Introduction: Cystic fibrosis is an autosomal recessive disorder caused by mutations in the
cystic fibrosis transmembrane conductance regulator (CFTR) gene that codes for the CFTR …

Cystic fibrosis (CF) is caused by the loss of the cystic fibrosis transmembrane conductance
regulator (CFTR) function and results in a respiratory phenotype that is characterized by …

In the respiratory system, Na+ absorption and Cl− secretion are balanced to maintain an
appropriate airway surface fluid (ASF) volume and ensure efficient mucociliary clearance. In …

Increased airway Na+ absorption mediated by the amiloride‐sensitive epithelial Na+
channel (ENaC) is a basic defect in cystic fibrosis (CF) lung disease. Cystic fibrosis is one of …

Na+ absorption and Cl− secretion are in equilibrium to maintain an appropriate airway
surface fluid volume and ensure appropriate mucociliary clearance. In cystic fibrosis, this …

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 …

Download: Download high-res image (36KB) Download: Download full-size image Cystic
fibrosis (CF) is a monogenic disease caused by mutations in the cystic fibrosis …

The epithelial sodium channel (ENaC) represents the rate-limiting step of sodium absorption
across airway epithelia and thereby constitutes the major pathway for volume absorption …

Airway surface hydration depends on the balance between transepithelial Na+ absorption
and Cl− secretion. In adult mice, absence of functional cystic fibrosis transmembrane …