Cystic fibrosis is a multiorgan disease caused by impaired function of the cystic fibrosis
transmembrane conductance regulator (CFTR). Since the introduction of the CFTR …

Pseudomonas aeruginosa opportunistically infects the airways of patients with cystic fibrosis
and causes significant morbidity and mortality. Initial infection can often be eradicated …

Background Abnormal macrophage function caused by dysfunctional cystic fibrosis
transmembrane conductance regulator (CFTR) is a critical contributor to chronic airway …

Highly-effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator
therapy (HEMT) corrects the underlying molecular defect causing cystic fibrosis (CF) …

Cystic fibrosis (CF), the most common lethal genetic disease in Caucasians, is characterized
by chronic bacterial lung infection and excessive inflammation, which lead to progressive …

Cystic fibrosis is caused by gene mutations that result in an abnormal Cystic Fibrosis
Transmembrane Conductance Regulator (CFTR) protein on the surface of cells. CFTR …

The preservation of cellular homeostasis requires the synthesis of new proteins
(proteostasis) and organelles, and the effective removal of misfolded or impaired proteins …

Background Ivacaftor has been shown to improve lung function and body weight in patients
with CF and a gating mutation. Real-world evaluation is warranted to examine its safety and …

Background CFTR modulators decrease some etiologies of CF airway inflammation;
however, data indicate that non-resolving airway infection and inflammation persist in …

Cystic fibrosis patients exhibit chronic Pseudomonas aeruginosa respiratory infections and
sustained proinflammatory state favoring lung tissue damage and remodeling, ultimately …