Mucus, mucins, and cystic fibrosis.
biochemical interactions
cftr
mucins
mucus
polymeric network
viscoelastic properties
Journal
Pediatric pulmonology
ISSN: 1099-0496
Titre abrégé: Pediatr Pulmonol
Pays: United States
ID NLM: 8510590
Informations de publication
Date de publication:
11 2019
11 2019
Historique:
received:
03
06
2019
accepted:
06
09
2019
entrez:
13
11
2019
pubmed:
13
11
2019
medline:
10
5
2020
Statut:
ppublish
Résumé
Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and is characterized by the accumulation of thick, adherent mucus plaques in multiple organs, of which the lungs, gastrointestinal tract and pancreatic ducts are the most commonly affected. A similar pathogenesis cascade is observed in all of these organs: loss of CFTR function leads to altered ion transport, consisting of decreased chloride and bicarbonate secretion via the CFTR channel and increased sodium absorption via epithelial sodium channel upregulation. Mucosa exposed to changes in ionic concentrations sustain severe pathophysiological consequences. Altered mucus biophysical properties and weakened innate defense mechanisms ensue, furthering the progression of the disease. Mucins, the high-molecular-weight glycoproteins responsible for the viscoelastic properties of the mucus, play a key role in the disease but the actual mechanism of mucus accumulation is still undetermined. Multiple hypotheses regarding the impact of CFTR malfunction on mucus have been proposed and are reviewed here. (a) Dehydration increases mucin monomer entanglement, (b) defective Ca
Identifiants
pubmed: 31715083
doi: 10.1002/ppul.24530
pmc: PMC6853602
mid: NIHMS1051245
doi:
Substances chimiques
CFTR protein, human
0
Mucins
0
Cystic Fibrosis Transmembrane Conductance Regulator
126880-72-6
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
S84-S96Subventions
Organisme : NHLBI NIH HHS
ID : 5 P01 HL 108808-08
Pays : United States
Organisme : NIDDK NIH HHS
ID : 5 P30 DK 065988-14
Pays : United States
Organisme : NHLBI NIH HHS
ID : P01 HL108808
Pays : United States
Organisme : NIDDK NIH HHS
ID : P30 DK065988
Pays : United States
Informations de copyright
© 2019 Wiley Periodicals, Inc.
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