Journal article
Elastic mucus strands impair mucociliary clearance in cystic fibrosis pigs
Proceedings of the National Academy of Sciences - PNAS, Vol.119(13), pp.e2121731119-e2121731119
03/29/2022
DOI: 10.1073/pnas.2121731119
PMCID: PMC9060506
PMID: 35324331
Abstract
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.
Details
- Title: Subtitle
- Elastic mucus strands impair mucociliary clearance in cystic fibrosis pigs
- Creators
- Maria I Pino-Argumedo - Roy J. and Lucille A. Carver College of MedicineAnthony J Fischer - University of IowaBrieanna M Hilkin - University of IowaNicholas D Gansemer - University of IowaPatrick D Allen - University of IowaEric A Hoffman - University of IowaDavid A Stoltz - University of IowaMichael J Welsh - University of IowaMahmoud H Abou Alaiwa - University of Iowa
- Resource Type
- Journal article
- Publication Details
- Proceedings of the National Academy of Sciences - PNAS, Vol.119(13), pp.e2121731119-e2121731119
- DOI
- 10.1073/pnas.2121731119
- PMID
- 35324331
- PMCID
- PMC9060506
- NLM abbreviation
- Proc Natl Acad Sci U S A
- ISSN
- 0027-8424
- eISSN
- 1091-6490
- Publisher
- National Academy of Sciences
- Grant note
- DOI: 10.13039/100000050, name: HHS | NIH | National Heart, Lung, and Blood Institute, award: K08 HL136927; DOI: 10.13039/100000897, name: Cystic Fibrosis Foundation, award: CFF FISCHE1610; DOI: 10.13039/100000050, name: HHS | NIH | National Heart, Lung, and Blood Institute, award: K08 HL135433; DOI: 10.13039/100000897, name: Cystic Fibrosis Foundation, award: CFF ABOU20A0-KB; DOI: 10.13039/100000897, name: Cystic Fibrosis Foundation, award: CFF STOLTZ19R0; DOI: 10.13039/100000897, name: Cystic Fibrosis Foundation, award: CFF STOLTZ16XX0; DOI: 10.13039/100000050, name: HHS | NIH | National Heart, Lung, and Blood Institute, award: R01 HL136813; DOI: 10.13039/100000050, name: HHS | NIH | National Heart, Lung, and Blood Institute, award: HL091842; DOI: 10.13039/100000050, name: HHS | NIH | National Heart, Lung, and Blood Institute, award: HL051670
- Language
- English
- Date published
- 03/29/2022
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Neurology; Radiology; Molecular Physiology and Biophysics; Pulmonary, Critical Care, and Occupational Medicine; Pulmonary Medicine; Stead Family Department of Pediatrics; Fraternal Order of Eagles Diabetes Research Center; Neurosurgery; Internal Medicine
- Record Identifier
- 9984259442002771
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