Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the
Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane …

Submucosal glands contribute to airway surface liquid (ASL), a film that protects all airway
surfaces. Glandular mucus comprises electrolytes, water, the gel-forming mucin MUC5B …

Mucus pathology in cystic fibrosis (CF) has been known for as long as the disease has been
recognized and is sometimes called mucoviscidosis. The disease is marked by mucus …

The clinical manifestations of cystic fibrosis (CF) result from dysfunction of the cystic fibrosis
transmembrane regulator protein (CFTR). The majority of people with CF have a limited life …

Without cystic fibrosis transmembrane conductance regulator–mediated (CFTR-mediated)
HCO3–secretion, airway epithelia of newborns with cystic fibrosis (CF) produce an …

The gene that encodes for the cystic fibrosis transmembrane regulator protein (CFTR) was
identified in 1989, yet major pathophysiologic questions remain unanswered. There is …

Cystic fibrosis is a hereditary disease that mainly affects secretory organs in humans. It is
caused by mutations in the gene encoding CFTR with the most common phenylalanine …

The airway surface liquid (ASL) is a thin sheet of fluid that covers the luminal aspect of the
airway epithelium. The ASL is a site of several first-line host defenses, and its composition is …

Background Balanced activation and inhibition of the immune system ensures pathogen
clearance while avoiding hyperinflammation. Siglecs, sialic acid–binding proteins found on …

The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-regulated anion
channel, which is expressed on the apical plasma membrane (PM) of epithelial cells …