[HTML][HTML] New generation ENaC inhibitors detach cystic fibrosis airway mucus bundles via sodium/hydrogen exchanger inhibition

M Giorgetti, N Klymiuk, A Bähr, M Hemmerling… - European journal of …, 2021 - Elsevier
… 2A), we analyzed the mucus detaching properties of new generation ENaC inhibitors in
mouse ileum. Compound A was previously evaluated for its ability to inhibit ENaC (Åstrand et al., …

[HTML][HTML] Acidic submucosal gland pH and elevated protein concentration produce abnormal cystic fibrosis mucus

Y Xie, L Lu, XX Tang, TO Moninger, TJ Huang… - Developmental cell, 2020 - cell.com
… In cystic fibrosis (CF) pigs, loss of cystic fibrosis transmembrane conductance regulator (CFTR)
anion channels produced submucosal gland mucus that … these variables alter mucus, we …

Mucus concentration–dependent biophysical abnormalities unify submucosal gland and superficial airway dysfunction in cystic fibrosis

T Kato, G Radicioni, MJ Papanikolas, GV Stoychev… - Science …, 2022 - science.org
… Because SMG mucus in naïve porcine models … mucus layer (12–15), we investigated whether
strand formation was unique to SMG mucus, ie, not a feature of superficial epithelial mucus

Treatment of cystic fibrosis airway cells with CFTR modulators reverses aberrant mucus properties via hydration

CB Morrison, KM Shaffer, KC Araba… - European …, 2022 - Eur Respiratory Soc
… Question Cystic fibrosis (CF) is characterised by the accumulation of viscous adherent
mucus in the lungs. While several hypotheses invoke a direct relationship with cystic fibrosis

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

C Ehre, GC Hansson, DJ Thornton… - … of Cystic Fibrosis, 2023 - Elsevier
… In CF, dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) … of
mucus, as well as bacterial killing. As a result of altered mucus properties, mucus detachment

Elastic mucus strands impair mucociliary clearance in cystic fibrosis pigs

MI Pino-Argumedo, AJ Fischer… - Proceedings of the …, 2022 - National Acad Sciences
… MCT is defective in cystic fibrosis (CF), a life-threatening genetic disease caused by
mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) …

Physiology and pathophysiology of human airway mucus

DB Hill, B Button, M Rubinstein… - Physiological …, 2022 - journals.physiology.org
mucus in muco-obstructive lung diseases, eg, cystic fibrosis (CF), chronic obstructive pulmonary
… compression of the mucus layer onto the airway surface with the formation of adherent …

[HTML][HTML] Mucins and CFTR: their close relationship

K Okuda, KM Shaffer, C Ehre - International journal of molecular sciences, 2022 - mdpi.com
Cystic fibrosis (CF), an autosomal genetic disorder caused by the dysfunction of the cystic
fibrosis … failed mucociliary clearance due to abnormal mucus biophysical properties. In recent …

Mucosal immunity in cystic fibrosis

CM Bojanowski, S Lu, JK Kolls - The Journal of Immunology, 2021 - journals.aai.org
The highly complex and variable genotype–phenotype relationships observed in cystic fibrosis
(CF) have been an area of growing interest since the discovery of the CF transmembrane …

[HTML][HTML] Mucus, microbiomes and pulmonary disease

OW Meldrum, SH Chotirmall - Biomedicines, 2021 - mdpi.com
… and discuss the properties, function, and clinical translational relevance of pulmonary mucus
and assess it as a potential treatment target in pneumonia and chronic pulmonary disease. …