Open AccessElastic mucus strands impair mucociliary clearance in cystic fibrosis pigs
Author(s) -
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
Publication year - 2022
Publication title -
proceedings of the national academy of sciences of the united states of america
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.011
H-Index - 771
eISSN - 1091-6490
pISSN - 0027-8424
DOI - 10.1073/pnas.2121731119
Subject(s) - mucus , mucociliary clearance , cystic fibrosis , mucin , lung , cilium , pathology , chemistry , submucosal glands , medicine , anatomy , biology , microbiology and biotechnology , ecology
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.