The mucus clearance system is the dominant mechanical host defense system of the human
lung. Mucus is cleared from the lung by cilia and airflow, including both two-phase gas-liquid …

Mucus covers all wet epithelia and acts as a protective barrier. In the airways of the lungs,
the viscoelastic mucus meshwork entraps and clears inhaled materials and efficiently …

Mucociliary clearance is a critical defense mechanism for the lungs governed by regionally
coordinated epithelial cellular activities, including mucin secretion, cilia beating, and …

Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations,
affecting around 50,000 patients in Europe and 30,000 in United States. A mutation in CF …

After more than two decades since clinical trials tested the first use of recombinant adeno-
associated virus (rAAV) to treat cystic fibrosis (CF) lung disease, gene therapy for this …

HCO3− secretion in distal airways is critical for airway mucosal defense. HCO3−/H+
transport across the apical membrane of airway surface epithelial cells was studied by …

Bicarbonate secretion is a fundamental process involved in maintaining acid-base
homeostasis. Disruption of bicarbonate entry into airway lumen, as has been observed in …

Hypoxic Pulmonary Vasoconstriction (HPV) is an important physiological mechanism of the
lungs that matches perfusion to ventilation thus maximizing O2 saturation of the venous …

Simple Summary Cystic fibrosis (CF) is a multi-organ disease that affects the epithelia and
exocrine glands, particularly the lungs, but also the pancreas, liver, kidneys, and intestines …

Highlights•Analyzing CFTR mutations in patients with cystic fibrosis (CF).•Predicting drug
response for CF patients via organoid assays.•Modulating mutant CFTR activity with CF …