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 …

Cystic fibrosis (CF) is caused by mutations in the gene that encodes the cystic fibrosis
transmembrane conductance regulator (CFTR) anion channel. In humans and pigs, the loss …

Mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator
(CFTR) epithelial anion channel cause cystic fibrosis (CF). The multidomain integral …

Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the
Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane …

Neutrophil extracellular traps (NETs) are beneficial antimicrobial defense structures that can
help fight against invading pathogens in the host. However, recent studies reveal that NETs …

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 …

Knowledge on the mechanisms of acid and base secretion in airways has progressed
recently. The aim of this review is to summarize the known mechanisms of airway surface …

The pulmonary airways are continuously exposed to bacteria. As a first line of defense
against infection, the airway surface liquid (ASL) contains a complex mixture of antimicrobial …

Cystic fibrosis (CF) is a recessive genetic disease that is characterised by airway mucus
plugging and reduced mucus clearance. There are currently alternative hypotheses that …

The use of simulated biological fluids (SBFs) is a promising in vitro technique to better
understand the release mechanisms and possible in vivo behaviour of materials, including …