[HTML][HTML] Combined agonists act synergistically to increase mucociliary clearance in a cystic fibrosis airway model

NS Joo, HJ Cho, M Shinbashi, JY Choi, CE Milla… - Scientific reports, 2021 - nature.com
Mucus clearance, a primary innate defense mechanism of airways, is defective in patients
with cystic fibrosis (CF) and CF animals. In previous work, the combination of a low dose of …

[HTML][HTML] Marked increases in mucociliary clearance produced by synergistic secretory agonists or inhibition of the epithelial sodium channel

NS Joo, JH Jeong, HJ Cho, JJ Wine - Scientific reports, 2016 - nature.com
Mucociliary clearance (MCC) is a critical host innate defense mechanism in airways, and it is
impaired in cystic fibrosis (CF) and other obstructive lung diseases. Epithelial fluid secretion …

The mucus bundles responsible for airway cleaning are retained in cystic fibrosis and by cholinergic stimulation

A Ermund, LN Meiss, B Dolan, A Bähr… - European …, 2018 - Eur Respiratory Soc
The beneficial effect of anticholinergic therapy for chronic lung diseases such as chronic
obstructive pulmonary disease (COPD) is well documented, although cholinergic stimulation …

Role of cilia, mucus, and airway surface liquid in mucociliary dysfunction: lessons from mouse models

MA Mall - Journal of aerosol medicine and pulmonary drug …, 2008 - liebertpub.com
Mucociliary clearance is an important primary innate defense mechanism that protects the
lungs from deleterious effects of inhaled pollutants, allergens, and pathogens. Mucociliary …

[HTML][HTML] Muc5b contributes to mucus abnormality in rat models of cystic fibrosis

JD Keith, AG Henderson, CM Fernandez-Petty… - Frontiers in …, 2022 - frontiersin.org
Cystic fibrosis (CF) airway disease is characterized by excessive and accumulative mucus in
the airways. Mucociliary clearance becomes defective as mucus secretions become …

Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice

JM Zahm, D Gaillard, F Dupuit… - … of Physiology-Cell …, 1997 - journals.physiology.org
In cystic fibrosis (CF), whether cystic fibrosis transmembrane conductance regulator (CFTR)
dysfunction leads to decreased mucociliary clearance and mucus hypersecretion, before …

Mucociliary Clearance is Impaired in Small Airways of Cystic Fibrosis Pigs

CG Stewart, BM Hilkin, ND Gansemer, DW Dick… - bioRxiv, 2024 - biorxiv.org
Rationale: Cystic fibrosis is a genetic disorder characterized by recurrent airway infections,
inflammation, and progressive decline in lung function. Autopsy and spirometry data suggest …

Mucociliary transport in healthy and cystic fibrosis pig airways

Y Xie, L Ostedgaard, MH Abou Alaiwa, L Lu… - Annals of the …, 2018 - atsjournals.org
Cystic fibrosis (CF) lung disease is the major cause of morbidity and mortality in people with
CF. Abnormal mucociliary transport has been the leading hypothesis for the underlying …

Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis

MJ Hoegger, AJ Fischer, JD McMenimen… - Science, 2014 - science.org
Lung disease in people with cystic fibrosis (CF) is initiated by defective host defense that
predisposes airways to bacterial infection. Advanced CF is characterized by a deficit in …

Tromethamine improves mucociliary clearance in cystic fibrosis pigs

JJ Ash, BM Hilkin, ND Gansemer… - Physiological …, 2022 - Wiley Online Library
In cystic fibrosis (CF), the loss of cystic fibrosis transmembrane conductance regulator
(CFTR) mediated Cl− and HCO3− secretion across the epithelium acidifies the airway …