Journal article
Mucus aberrant properties in CF: Insights from cells and animal models
Journal of cystic fibrosis, Vol.22(Suppl. 1), pp.S23-S26
03/2023
DOI: 10.1016/j.jcf.2022.08.019
PMCID: PMC10018425
PMID: 36117114
Abstract
Cystic fibrosis (CF), an autosomal genetic disorder caused by the dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, is characterized by mucus accumulation in the lungs, the intestinal tract, and the pancreatic ducts. Mucins are high-molecular-weight glycoproteins that govern the biochemical and biophysical properties of mucus. In the CF lung, increased mucus viscoelasticity is associated with decreased mucociliary clearance and defects in host defense mechanisms. The link between defective ion channel and abnormal mucus properties has been investigated in studies involving cell and animal models. In this review article, we discuss recent progress toward understanding the different regions and cells that express CFTR in the airways and how mucus is produced and cleared from the lungs. In addition, we reflect on animal models that provided insights into the organization and the role of the mucin network and how mucus and antimicrobial activities act in concert to protect the lungs from invading pathogens.
Details
- Title: Subtitle
- Mucus aberrant properties in CF: Insights from cells and animal models
- Creators
- Camille EhreGunnar C HanssonDavid J ThorntonLynda S Ostedgaard
- Resource Type
- Journal article
- Publication Details
- Journal of cystic fibrosis, Vol.22(Suppl. 1), pp.S23-S26
- DOI
- 10.1016/j.jcf.2022.08.019
- PMID
- 36117114
- PMCID
- PMC10018425
- NLM abbreviation
- J Cyst Fibros
- ISSN
- 1569-1993
- eISSN
- 1873-5010
- Grant note
- DOI: 10.13039/501100004359, name: Vetenskapsrådet, award: 2017-00958; DOI: 10.13039/100000002, name: National Institutes of Health, award: HL163556-01, PPG Hl091842-15; DOI: 10.13039/100000062, name: National Institute of Diabetes and Digestive and Kidney Diseases, award: 2 P30 DK 065988-16; DOI: 10.13039/100000897, name: Cystic Fibrosis Foundation, award: EHRE20XX0; DOI: 10.13039/501100000849, name: National Centre for the Replacement, Refinement and Reduction of Animals in Research; DOI: 10.13039/501100003793, name: Hjärt-Lungfonden, award: 20210377
- Language
- English
- Electronic publication date
- 09/15/2022
- Date published
- 03/2023
- Academic Unit
- Internal Medicine
- Record Identifier
- 9984359663102771
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