Neutrophil elastase (NE) is a major inflammatory protease released by neutrophils and is
present in the airways of patients with cystic fibrosis (CF), chronic obstructive pulmonary …

Surfactant proteins A (SP-A) and D (SP-D) are soluble innate immune molecules which
maintain lung homeostasis through their dual roles as anti-infectious and …

Background Recent studies demonstrated that the triple combination cystic fibrosis
transmembrane conductance regulator (CFTR) modulator therapy elexacaftor/tezacaftor …

With the 2019 breakthrough in the development of highly effective modulator therapy
providing unprecedented clinical benefits for over 90% of patients with cystic fibrosis who …

Neutrophils have a prominent role in all human immune responses against any type of
pathogen or stimulus. The lungs are a major neutrophil reservoir and neutrophilic …

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 …

Cystic fibrosis (CF) is the most common genetic disorder among Caucasians, estimated to
affect more than 70,000 people in the world. Severe and persistent bronchial inflammation …

Dysregulated protease activity has long been implicated in the pathogenesis of chronic lung
diseases and especially in conditions that display mucus obstruction, such as chronic …

A hallmark of cystic fibrosis (CF) chronic respiratory disease is an extensive neutrophil
infiltrate in the mucosa filling the bronchial lumen, starting early in life for CF infants. The …

The cystic fibrosis transmembrane conductance regulator (CFTR) is a crucial ion channel for
transport of chloride and bicarbonate anions. Functional roles of CFTR have been identified …