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 …

From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations

G Veit, RG Avramescu, AN Chiang… - Molecular biology of …, 2016 - Am Soc Cell Biol
More than 2000 mutations in the cystic fibrosis transmembrane conductance regulator
(CFTR) have been described that confer a range of molecular cell biological and functional …

Epithelial mesenchymal transition (EMT): a universal process in lung diseases with implications for cystic fibrosis pathophysiology

N Rout-Pitt, N Farrow, D Parsons, M Donnelley - Respiratory research, 2018 - Springer
Cystic Fibrosis (CF) is a genetic disorder that arises due to mutations in the Cystic Fibrosis
Transmembrane Conductance Regulator gene, which encodes for a protein responsible for …

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 …

A periciliary brush promotes the lung health by separating the mucus layer from airway epithelia

B Button, LH Cai, C Ehre, M Kesimer, DB Hill… - Science, 2012 - science.org
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 …

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

AA Pezzulo, XX Tang, MJ Hoegger, MH Abou Alaiwa… - Nature, 2012 - nature.com
Cystic fibrosis (CF) is a life-shortening disease caused by mutations in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene. Although bacterial lung infection and …

Current and future treatments in primary ciliary dyskinesia

T Paff, H Omran, KG Nielsen, EG Haarman - International journal of …, 2021 - mdpi.com
Primary ciliary dyskinesia (PCD) is a rare genetic ciliopathy in which mucociliary clearance
is disturbed by the abnormal motion of cilia or there is a severe reduction in the generation …

Mucus clearance as a primary innate defense mechanism for mammalian airways

MR Knowles, RC Boucher - The Journal of clinical …, 2002 - Am Soc Clin Investig
Pulmonary defense mechanisms preventing chronic bacterial infection. The lung is depicted
as an inverted funnel, reflecting the relative surface area of distal versus proximal airways …

Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients

D Worlitzsch, R Tarran, M Ulrich… - The Journal of …, 2002 - Am Soc Clin Investig
Current theories of CF pathogenesis predict different predisposing “local environmental”
conditions and sites of bacterial infection within CF airways. Here we show that, in CF …