Inhibition of protease–epithelial sodium channel signaling improves mucociliary function in cystic fibrosis airways

JA Reihill, B Walker, RA Hamilton… - American journal of …, 2016 - atsjournals.org
Rationale: In cystic fibrosis (CF) a reduction in airway surface liquid (ASL) height
compromises mucociliary clearance, favoring mucus plugging and chronic bacterial …

Lubiprostone activates non-CFTR-dependent respiratory epithelial chloride secretion in cystic fibrosis mice

KD MacDonald, KR McKenzie… - … of Physiology-Lung …, 2008 - journals.physiology.org
Periciliary fluid balance is maintained by the coordination of sodium and chloride channels
in the apical membranes of the airways. In the absence of the cystic fibrosis transmembrane …

Effect of airway clearance therapies on mucociliary clearance in adults with cystic fibrosis: A randomized controlled trial

A Trimble, K Zeman, J Wu, A Ceppe, W Bennett… - PloS one, 2022 - journals.plos.org
Background Cystic fibrosis (CF) is an inherited disorder causing impaired mucociliary
clearance within the respiratory tract, and is associated with bronchiectasis, chronic …

Resveratrol ameliorates abnormalities of fluid and electrolyte secretion in a hypoxia‐Induced model of acquired CFTR deficiency

BA Woodworth - The Laryngoscope, 2015 - Wiley Online Library
Objective/Hypothesis Ineffective mucociliary clearance (MCC) is a common
pathophysiologic process that underlies airway inflammation and infection. A dominant fluid …

A new paradigm in respiratory hygiene: modulating respiratory secretions to contain cough bioaerosol without affecting mucus clearance

G Zayas, JC Valle, M Alonso, H Alfaro, D Vega… - BMC Pulmonary …, 2007 - Springer
Background Several strategies and devices have been designed to protect health care
providers from acquiring transmissible respiratory diseases while providing care. In …

[引用][C] P273 BI 443651 and BI 1265162 demonstrate in vitro inhibition of epithelial sodium channel (ENaC) in the Ussing chamber

B Jung, P Iacono, K Benediktus, M Hahn… - Journal of Cystic …, 2019 - Elsevier

Mucus aberrant properties in CF: Insights from cells and animal models

C Ehre, GC Hansson, DJ Thornton… - Journal of Cystic …, 2023 - Elsevier
Cystic fibrosis (CF), an autosomal genetic disorder caused by the dysfunction of the cystic
fibrosis transmembrane conductance regulator (CFTR) protein, is characterized by mucus …

[引用][C] P275 Both BI 443651 and BI 1265162 demonstrate inhibition of the liquid absorption from the rat airway epithelium in vivo

B Jung, P Iacono, M Hahn, M Borsch, A Hoffmann… - Journal of Cystic …, 2019 - Elsevier

Pathological mucus and impaired mucus clearance in cystic fibrosis patients result from increased concentration, not altered pH

DB Hill, RF Long, WJ Kissner, E Atieh… - European …, 2018 - Eur Respiratory Soc
Cystic fibrosis (CF) is a recessive genetic disease that is characterised by airway mucus
plugging and reduced mucus clearance. There are currently alternative hypotheses that …

Mucociliary and cough clearance as a biomarker for therapeutic development

WD Bennett, E Daviskas, A Hasani… - Journal of aerosol …, 2010 - liebertpub.com
A workshop/symposium on “Mucociliary and Cough Clearance (MCC/CC) as a Biomarker
for Therapeutic Development” was held on October 21–22, 2008, in Research Triangle Park …