Pulmonary defense mechanisms preventing chronic bacterial infection. The lung is depicted
as an inverted funnel, reflecting the relative surface area of distal versus proximal airways …

Cystic fibrosis (CF) was distinguished from celiac disease in 1938. Then, it was a pathologic
diagnosis, life expectancy was approximately 6 months, and the autosomal recessive …

Rationale: The CFTR (cystic fibrosis transmembrane conductance regulator) modulator
combination elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was shown to improve clinical …

Cigarette smoke first interacts with the lung through the cellularly diverse airway epithelium
and goes on to drive development of most chronic lung diseases. Here, through single cell …

Cystic fibrosis (CF) is a fatal genetic disease caused by mutations in the gene encoding the
CF transmembrane conductance regulator (CFTR), a protein kinase A (PKA)-activated …

Background Ataluren was developed to restore functional protein production in genetic
disorders caused by nonsense mutations, which are the cause of cystic fibrosis in 10% of …

Cystic fibrosis (CF) remains the most common fatal hereditary lung disease. The discovery of
the cystic fibrosis transmembrane conductance regulator (CFTR) gene 25 years ago set the …

The apical (outward-facing) membranes of high-resistance epithelia contain Na+ channels,
traditionally identified by their sensitivity to block by the K (+)-sparing diuretic amiloride …

Cystic fibrosis is the most common potentially lethal autosomal recessive disease of
Caucasians, affecting 1 in 2500 newborns. Since the recent identification of the gene that is …

Cystic fibrosis is the most common fatal genetic disease affecting Caucasians and is
perhaps best characterized as an exocrinopathy involving a disturbance in fluid and …