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 …

Mucus, mucins, and cystic fibrosis

CB Morrison, MR Markovetz, C Ehre - Pediatric pulmonology, 2019 - Wiley Online Library
Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the
Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane …

Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on sputum viscoelastic properties, airway infection and inflammation in patients with cystic fibrosis

L Schaupp, A Addante, M Völler… - European …, 2023 - Eur Respiratory Soc
Background Recent studies demonstrated that the triple combination cystic fibrosis
transmembrane conductance regulator (CFTR) modulator therapy elexacaftor/tezacaftor …

[HTML][HTML] IL-1β dominates the promucin secretory cytokine profile in cystic fibrosis

G Chen, L Sun, T Kato, K Okuda… - The Journal of …, 2019 - Am Soc Clin Investig
Cystic fibrosis (CF) lung disease is characterized by early and persistent mucus
accumulation and neutrophilic inflammation in the distal airways. Identification of the factors …

TMEM16A potentiation: a novel therapeutic approach for the treatment of cystic fibrosis

HL Danahay, S Lilley, R Fox, H Charlton… - American journal of …, 2020 - atsjournals.org
Rationale: Enhancing non–CFTR (cystic fibrosis transmembrane conductance regulator)-
mediated anion secretion is an attractive therapeutic approach for the treatment of cystic …

Progression of cystic fibrosis lung disease from childhood to adulthood: neutrophils, neutrophil extracellular trap (NET) formation, and NET degradation

MA Khan, ZS Ali, N Sweezey, H Grasemann… - Genes, 2019 - mdpi.com
Genetic defects in cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gene
cause CF. Infants with CFTR mutations show a peribronchial neutrophil infiltration prior to …

Polymeric nitric oxide delivery nanoplatforms for treating cancer, cardiovascular diseases, and infection

G Jin, Z Gao, Y Liu, J Zhao, H Ou… - Advanced Healthcare …, 2021 - Wiley Online Library
The shortened Abstract is as follows: Therapeutic gas nitric oxide (NO) has demonstrated
the unique advances in biomedical applications due to its prominent role in regulating …

Airway mucus hyperconcentration in non–cystic fibrosis bronchiectasis

KA Ramsey, ACH Chen, G Radicioni… - American journal of …, 2020 - atsjournals.org
Rationale: Non–cystic fibrosis bronchiectasis is characterized by airway mucus
accumulation and sputum production, but the role of mucus concentration in the …

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 …

The MUC5B mucin polymer is dominated by repeating structural motifs and its topology is regulated by calcium and pH

GW Hughes, C Ridley, R Collins, A Roseman, R Ford… - Scientific reports, 2019 - nature.com
The polymeric mucin MUC5B provides the structural and functional framework of respiratory
mucus, conferring both viscoelastic and antimicrobial properties onto this vital protective …