Mucus, mucins, and cystic fibrosis

CB Morrison, MR Markovetz, C Ehre - Pediatric pulmonology, 2019 - Wiley Online Library
… Development of an airway mucus defect in the cystic fibrosis rat. JCI Insight. 2018;3(1):…
Molecular basis for pHdependent mucosal dehydration in cystic fibrosis airways. Proc Natl Acad …

[HTML][HTML] Airway surface liquid pH regulation in airway epithelium current understandings and gaps in knowledge

M Zajac, E Dreano, A Edwards, G Planelles… - … journal of molecular …, 2021 - mdpi.com
… Chloride secretion mainly involves the cystic fibrosis … the epithelium against dehydration
and bathes the cilia [29]. … the pH-dependent color changes of organic dye molecules (…

[HTML][HTML] Inflammation as a regulator of the airway surface liquid pH in cystic fibrosis

T Rehman, MJ Welsh - Cells, 2023 - mdpi.com
… the cellular and molecular mechanisms that control pH ASL . … an airway phenotype marked
by thick, adherent mucus and … viruses into airway epithelial cells (eg, pH-dependent entry of …

[HTML][HTML] Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis

J Simonin, E Bille, G Crambert, S Noel, E Dreano… - Scientific reports, 2019 - nature.com
… , with prolonged time of contact and continuous reseeding from infected mucus plugs. It is …
that this impaired host defense is pH-dependent. Increasing CF ASL pH improved its …

Physiology and pathophysiology of human airway mucus

DB Hill, B Button, M Rubinstein… - Physiological …, 2022 - journals.physiology.org
mechanisms derange the ion transport pathways that normally hydrate mucus in muco-obstructive
lung diseases, eg, cystic fibrosis … prevent dehydration by the osmotically active mucus

Inhibition of the sodium-dependent HCO3-transporter SLC4A4, produces a cystic fibrosis-like airway disease phenotype

V Saint-Criq, A Guequén, AR Philp, S Villanueva… - Elife, 2022 - elifesciences.org
… entry into airway lumen, as has been observed in cystic fibrosis, produces several defects in
lung function due to thick mucusMolecular basis for pH- dependent mucosal dehydration in …

Small-molecule ion channels increase host defences in cystic fibrosis airway epithelia

KA Muraglia, RS Chorghade, BR Kim, XX Tang… - Nature, 2019 - nature.com
airway surface liquid pH, viscosity, and antibacterial activity in primary cultures of airway
epithelia from people with cystic fibrosis … yield CFTR, and increased airway surface liquid pH in …

[HTML][HTML] A glycopolymer improves vascoelasticity and mucociliary transport of abnormal cystic fibrosis mucus

CM Fernandez-Petty, GW Hughes, HL Bowers… - Jci Insight, 2019 - ncbi.nlm.nih.gov
Cystic fibrosis (CF) is characterized by increased mucus viscosity and delayed … Mucus in
the respiratory and GI tract is excessively adhesive in the presence of airway dehydration and …

[HTML][HTML] CFTR protein: not just a chloride channel?

LS Hanssens, J Duchateau, GJ Casimir - Cells, 2021 - mdpi.com
Cystic fibrosis (CF) is a recessive genetic disease caused by … This bactericidal activity of
sphingosine itself is also pH dependentAirway obstruction in CF is due to mucus dehydration/…

[HTML][HTML] Airway surface hyperviscosity and defective mucociliary transport by IL-17/TNF-α are corrected by β-adrenergic stimulus

D Guidone, M Buccirossi, P Scudieri, M Genovese… - JCI insight, 2022 - ncbi.nlm.nih.gov
cystic fibrosis (CF), loss of CFTR chloride channel function causes airway surface dehydration
activity of antimicrobial molecules and alter the properties and the release of mucus (13–16…