Mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator
undermine many host defense systems by inhibiting the function of airway-surface liquid …

Inflammation plays a critical role in cystic fibrosis (CF) lung pathology and disease
progression making it an active area of research and important therapeutic target. In this …

Background Ivacaftor is the first in a class of drugs, CFTR modulators, that target the
underlying defect in cystic fibrosis (CF). This long-term observational safety study evaluated …

Background Cystic fibrosis (CF) is a multisystem disease with progressive deterioration.
Recently, CF transmembrane conductance regulator (CFTR) modulator therapies were …

Lung disease causes most of the morbidity and mortality in cystic fibrosis (CF).
Understanding the pathogenesis of this disease has been hindered, however, by the lack of …

Studies of the biofilm life cycle can identify novel targets and strategies for improving biofilm
control measures. Of particular interest are dispersal events, where a subpopulation of cells …

The host inflammatory response in cystic fibrosis (CF) lung disease has long been
recognized as a central pathological feature and an important therapeutic target. Indeed …

Despite significant advances in treatment strategies targeting the underlying defect in cystic
fibrosis (CF), airway infection remains an important cause of lung disease. In this two-part …

The opportunistic human pathogen Pseudomonas aeruginosa is the predominant micro-
organism of chronic lung infections in cystic fibrosis patients. P. aeruginosa colonizes the …

Cystic fibrosis (CF) is a common, life-threatening, multisystemic, autosomal recessive
disorder. In the last few years, giant steps have been made with regard to the understanding …