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 …

Standard follow-up and symptomatic treatment have allowed most patients with cystic
fibrosis to live to young adulthood. However, many patients still die prematurely from …

Salt and fluid absorption and secretion are two processes that are fundamental to epithelial
function and whole body fluid homeostasis, and as such are tightly regulated in epithelial …

Cystic fibrosis (CF) is a recessive genetic disease caused by mutations in a gene encoding
a protein called Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). The CFTR …

Inflammation plays a critical role in cystic fibrosis (CF) lung pathology and disease
progression making it an active area of research and important therapeutic target. In this …

The airway epithelium is now considered to be central to the orchestration of pulmonary
inflammatory and immune responses, and is also key to tissue remodelling. It acts as the first …

Mucus clearance is the primary defense mechanism that protects airways from inhaled
infectious and toxic agents. In the current gel-on-liquid mucus clearance model, a mucus gel …

Pulmonary defense against environmental injury depends on airway mucus, which traps
inhaled toxins that are then cleared from the lungs by ciliary beating and cough. This review …

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 …

Cystic fibrosis (CF) remains the most common life-shortening hereditary disease in white
populations, with high morbidity and mortality related to chronic airway mucus obstruction …