Vacuolar H+-ATPases are ubiquitous multisubunit complexes mediating the ATP-dependent
transport of protons. In addition to their role in acidifying the lumen of various intracellular …

Gel-forming mucins, the primary macromolecular components of airway mucus, facilitate
airway clearance by mucociliary transport. In cystic fibrosis (CF) altered mucus properties …

The mechanisms underlying mucus-associated pathologies in cystic fibrosis (CF) remain
obscure. However, recent studies indicate that CF transmembrane conductance regulator …

The pancreas is one of the earliest, and most commonly affected, organs in patients with
cystic fibrosis (CF). Studying the pathogenesis of pancreatic disease is limited in CF …

The ability to maintain proper airway surface liquid (ASL) volume homeostasis is vital for
mucus hydration and clearance, which are essential aspects of the mammalian lung's innate …

Defective transepithelial electrolyte transport is thought to initiate cystic fibrosis (CF) lung
disease. Yet, how loss of CFTR affects electrolyte transport remains uncertain. CFTR−/− pigs …

Aberrant HCO 3− transport is a hallmark of cystic fibrosis (CF) and is associated with
aberrant Cl−-dependent HCO 3− transport by the cystic fibrosis transmembrane …

Corneal transparency is dependent on regulation of the hydration of the corneal stroma.
Water is driven into the cornea across the epithelial and endothelial cell layers by the …

Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the gene
encoding the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel …

Background: The loss of cystic fibrosis transmembrane conductance regulator (CFTR)
mediated chloride conductance does not fully explain the diverse pathologies evident in …