Elastic mucus strands impair mucociliary clearance in cystic fibrosis pigs.
cystic fibrosis
epithelia
lung
mucus
submucosal gland
Journal
Proceedings of the National Academy of Sciences of the United States of America
ISSN: 1091-6490
Titre abrégé: Proc Natl Acad Sci U S A
Pays: United States
ID NLM: 7505876
Informations de publication
Date de publication:
29 03 2022
29 03 2022
Historique:
entrez:
24
3
2022
pubmed:
25
3
2022
medline:
4
5
2022
Statut:
ppublish
Résumé
SignificanceIn many lung diseases, increased amounts of and/or abnormal mucus impair mucociliary clearance, a key defense against inhaled and aspirated material. Submucosal glands lining cartilaginous airways secrete mucus strands that are pulled by cilia until they break free from the duct and sweep upward toward the larynx, carrying particulates. In cystic fibrosis (CF) pigs, progressive clearance of insufflated microdisks was repeatedly interrupted as microdisks abruptly recoiled. Aerosolizing a reducing agent to break disulfide bonds linking mucins ruptured mucus strands, freeing them from submucosal gland ducts and allowing cilia to propel them up the airways. These findings highlight the abnormally increased elasticity of CF mucus and suggest that agents that break disulfide bonds might have value in lung diseases with increased mucus.
Identifiants
pubmed: 35324331
doi: 10.1073/pnas.2121731119
pmc: PMC9060506
doi:
Substances chimiques
Disulfides
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
e2121731119Subventions
Organisme : NHLBI NIH HHS
ID : K08 HL135433
Pays : United States
Organisme : NHLBI NIH HHS
ID : P01 HL091842
Pays : United States
Organisme : NHLBI NIH HHS
ID : P01 HL152960
Pays : United States
Organisme : NHLBI NIH HHS
ID : R56 HL147073
Pays : United States
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