Physiology and pathophysiology of human airway mucus

DB Hill, B Button, M Rubinstein… - Physiological …, 2022 - journals.physiology.org
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 …

[HTML][HTML] Physicochemical properties of mucus and their impact on transmucosal drug delivery

J Leal, HDC Smyth, D Ghosh - International journal of pharmaceutics, 2017 - Elsevier
Mucus is a selective barrier to particles and molecules, preventing penetration to the
epithelial surface of mucosal tissues. Significant advances in transmucosal drug delivery …

Progress in therapies for cystic fibrosis

K De Boeck, MD Amaral - The Lancet Respiratory Medicine, 2016 - thelancet.com
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 …

Epithelial barrier dysfunction in chronic respiratory diseases

FM Carlier, C de Fays, C Pilette - Frontiers in physiology, 2021 - frontiersin.org
Mucosal surfaces are lined by epithelial cells, which provide a complex and adaptive
module that ensures first-line defense against external toxics, irritants, antigens, and …

Chronic rhinosinusitis pathogenesis

WW Stevens, RJ Lee, RP Schleimer… - Journal of Allergy and …, 2015 - Elsevier
There are a variety of medical conditions associated with chronic sinonasal inflammation,
including chronic rhinosinusitis (CRS) and cystic fibrosis. In particular, CRS can be divided …

Cystic fibrosis: emergence of highly effective targeted therapeutics and potential clinical implications

MA Mall, N Mayer-Hamblett… - American journal of …, 2020 - atsjournals.org
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 …

Ion channel modulators in cystic fibrosis

M Gentzsch, MA Mall - Chest, 2018 - Elsevier
Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR) gene and remains one of the most common life-shortening genetic …

Comparison of lung clearance index and magnetic resonance imaging for assessment of lung disease in children with cystic fibrosis

M Stahl, MO Wielpütz, SY Graeber… - American journal of …, 2017 - atsjournals.org
Rationale: Early onset and progression of lung disease in children with cystic fibrosis (CF)
indicates that sensitive noninvasive outcome measures are needed for diagnostic …

Cryo-EM structures and functional characterization of murine Slc26a9 reveal mechanism of uncoupled chloride transport

JD Walter, M Sawicka, R Dutzler - Elife, 2019 - elifesciences.org
The epithelial anion transporter SLC26A9 contributes to airway surface hydration and
gastric acid production. Colocalizing with CFTR, SLC26A9 has been proposed as a target …

Airway mucus, inflammation and remodeling: emerging links in the pathogenesis of chronic lung diseases

Z Zhou-Suckow, J Duerr, M Hagner, R Agrawal… - Cell and tissue …, 2017 - Springer
Airway mucus obstruction is a hallmark of many chronic lung diseases including rare genetic
disorders such as cystic fibrosis (CF) and primary ciliary dyskinesia, as well as common lung …