The arrival of cystic fibrosis transmembrane conductance regulator (CFTR) modulators as a
new class of treatment for cystic fibrosis (CF) in 2012 represented a pivotal advance in …

Prior studies have shown that fluid secretions from airway submucosal glands in cystic
fibrosis (CF) are reduced and hyperviscous, possibly contributing to the pathogenesis of CF …

Cystic fibrosis (CF) is a complex inherited disease which affects many organs, including the
pancreas and liver, gastrointestinal tract and reproductive system, sweat glands and …

Dysregulated expression and activity of cathepsin S (CTSS), a lysosomal protease and a
member of the cysteine cathepsin protease family, is linked to the pathogenesis of multiple …

The development of cystic fibrosis-related diabetes (CFRD) often leads to poorer outcomes
in patients with cystic fibrosis including increases in pulmonary exacerbations, poorer lung …

In cystic fibrosis (CF), defects in the CF transmembrane conductance regulator (CFTR)
channel lead to an acidic airway surface liquid (ASL), which compromises innate defence …

Lung-lining fluid (LLF) is a primary constituent of the pulmonary host defense system. It is
distributed continuously throughout the respiratory tract but is heterogeneous regarding its …

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 …

SCN−(thiocyanate) is an important physiological anion involved in innate defense of
mucosal surfaces. SCN− is oxidized by H 2 O 2, a reaction catalyzed by lactoperoxidase, to …

Importance of the field: CF airway mucus can be infected by opportunistic microorganisms,
notably Pseudomonas aeruginosa. Once organisms are established as biofilms, even the …