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 …

Cilia and mucociliary clearance

XM Bustamante-Marin… - Cold Spring Harbor …, 2017 - cshperspectives.cshlp.org
Mucociliary clearance (MCC) is the primary innate defense mechanism of the lung. The
functional components are the protective mucous layer, the airway surface liquid layer, and …

Secretory cells dominate airway CFTR expression and function in human airway superficial epithelia

K Okuda, H Dang, Y Kobayashi, G Carraro… - American journal of …, 2021 - atsjournals.org
Rationale: Identification of the specific cell types expressing CFTR (cystic fibrosis [CF]
transmembrane conductance regulator) is required for precision medicine therapies for CF …

Muco-obstructive lung diseases

RC Boucher - New England Journal of Medicine, 2019 - Mass Medical Soc
Muco-Obstructive Lung Diseases This review covers the normal mechanisms of mucus
formation and how they are abnormal in common conditions—such as COPD, cystic fibrosis …

CFTR: cystic fibrosis and beyond

MA Mall, D Hartl - 2014 - Eur Respiratory Soc
Cystic fibrosis (CF) remains the most common fatal hereditary lung disease. The discovery of
the cystic fibrosis transmembrane conductance regulator (CFTR) gene 25 years ago set the …

Mucins, mucus, and sputum

JA Voynow, BK Rubin - Chest, 2009 - Elsevier
Normal airway mucus lines the epithelial surface and provides an important innate immune
function by detoxifying noxious molecules and by trapping and removing pathogens and …

[HTML][HTML] Mucus clearance and lung function in cystic fibrosis with hypertonic saline

SH Donaldson, WD Bennett, KL Zeman… - … England Journal of …, 2006 - Mass Medical Soc
Background Abnormal homeostasis of the volume of airway surface liquid in patients with
cystic fibrosis is thought to produce defects in mucus clearance and airway defense …

[HTML][HTML] CFTR modulators: shedding light on precision medicine for cystic fibrosis

M Lopes-Pacheco - Frontiers in pharmacology, 2016 - frontiersin.org
Cystic fibrosis (CF) is the most common life-threatening monogenic disease afflicting
Caucasian people. It affects the respiratory, gastrointestinal, glandular and reproductive …

Airway surface dehydration in cystic fibrosis: pathogenesis and therapy

RC Boucher - Annu. Rev. Med., 2007 - annualreviews.org
Cystic fibrosis (CF) lung disease reflects the failure of airways defense against chronic
bacterial infection. Studies of CF cultures, transgenic mice, and CF patients suggest that the …

[HTML][HTML] Measurements of deposition, lung surface area and lung fluid for simulation of inhaled compounds

E Fröhlich, A Mercuri, S Wu… - Frontiers in …, 2016 - frontiersin.org
Modern strategies in drug development employ in silico techniques in the design of
compounds as well as estimations of pharmacokinetics, pharmacodynamics and toxicity …