Cystic fibrosis (CF) is a lethal inherited disease caused by mutations in the CF
transmembrane conductance regulator (CFTR) gene, which result in impairment of CFTR …

Cystic fibrosis (CF) is the most common life-threatening monogenic disease afflicting
Caucasian people. It affects the respiratory, gastrointestinal, glandular and reproductive …

Background Lung infections are among the most consequential manifestations of cystic
fibrosis (CF) and are associated with reduced lung function and shortened survival. Drugs …

Rationale: Previous work indicates that ivacaftor improves cystic fibrosis transmembrane
conductance regulator (CFTR) activity and lung function in people with cystic fibrosis and …

Background Cystic fibrosis (CF) lung disease is defined by large numbers of neutrophils and
associated damaging products in the airway. Delayed neutrophil apoptosis is described in …

Airway structure and function are key aspects of normal lung development, growth, and
aging, as well as of lung responses to the environment and the pathophysiology of important …

Cystic fibrosis results from mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR) anion channel, ultimately leading to diminished transepithelial anion …

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

Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed ubiquitously
throughout the body. Thus, while respiratory manifestations dominate much of cystic fibrosis …

Persistent activation of the transcription factor Nuclear factor-κB (NF-κB) is central to the
pathogenesis of many inflammatory disorders, including those of the lung such as cystic …