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Elastic mucus strands impair mucociliary clearance in cystic fibrosis pigs

2022; National Academy of Sciences; Volume: 119; Issue: 13 Linguagem: Inglês

10.1073/pnas.2121731119

1091-6490

Maria I. Pino-Argumedo, Anthony J. Fischer, Brieanna M. Hilkin, Nicholas D. Gansemer, Patrick D. Allen, Eric A. Hoffman, David A. Stoltz, Michael J. Welsh, Mahmoud H. Abou Alaiwa,

Inhalation and Respiratory Drug Delivery

Significance In 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.