Molecular defects in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR)
gene are well understood. Despite this, how defects in the CFTR lead to CF is not clear. This …

Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis
transmembrane conductance regulator (CFTR) channel, which can result in chronic lung …

The airway is kept sterile by an efficient innate defense mechanism. The cornerstone of
airway defense is mucus containing diverse antimicrobial factors that kill or inactivate …

Airway submucosal glands have been proposed as a primary site for initiating and
sustaining airway disease in cystic fibrosis (CF). However, it has been difficult to define the …

Dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) anion
channel by genetic mutations causes the inherited disease cystic fibrosis (CF). CF lung …

Submucosal glands line the cartilaginous airways and produce most of the antimicrobial
mucus that keeps the airways sterile. The glands are defective in cystic fibrosis (CF), but how …

Rationale: development, little is known about how the lack of cystic fibrosis transmembrane
conductance regulator (CFTR) function contributes to disease pathogenesis in small …

Cystic fibrosis (CF) is an inherited disorder causing chronic airway obstruction and
inflammation in the lungs which ultimately lead to respiratory failure and death. CF arises …

Pathological and clinical data suggest that distal small airways, with a diameter less than 2
mm, play important roles in the pathogenesis of cystic fibrosis (CF) lung disease. Lack of …