Mucin Secretion in Cystic Fibrosis: A Systematic Review.
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TLDR
Increased expression of MUC5AC in the surface epithelium and of M UC5B in the subepithelial glands may be the result of higher secretion rate of mucin into the lumen of the respiratory tract, causing mucus plaque, infection, and inflammation.Abstract:
Background Mucus protects the epithelium against invaders and toxic materials. Sticky and thick mucus is characteristic of CF. Objective The aim of this systematic review is to characterize the specific mucins secreted in the lung and intestinal tract of CF patients. Methods A systematic literature search was conducted up to December 31, 2019. The following terms were used: "cystic fibrosis" AND "mucin." Case-control studies comparing mucin expression in CF patients to healthy controls were included. Results We found 741 eligible studies, 694 studies were rejected because they were performed in animals and not in full text, and 32 studies were excluded being editorials, duplications, review articles, meta-analysis, or not in English. Fifteen studies were eligible for our study, including 150 CF patients compared to 82 healthy controls, all fulfilled the inclusion criteria. The main mucin types expressed in the sinus submucosal glands, sputum, tracheobronchial surface epithelium, and lung submucosal glands were MUC5AC and MUC5B. Increase in the number of sinusoidal submucosal glands and expression of MUC5B was found in CF patients, but no such difference from healthy controls was found for the number of goblet cells in the surface epithelium nor in the expression of -MUC5AC. The opposite was found in the tracheobronchial surface epithelium and in the lungs. Conclusions Increased expression of MUC5AC in the surface epithelium and of MUC5B in the subepithelial glands may be the result of higher secretion rate of mucin into the lumen of the respiratory tract, causing mucus plaque, infection, and inflammation.read more
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References
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Mucins and mucosal protection in the gastrointestinal tract: new prospects for mucins in the pathology of gastrointestinal disease
TL;DR: Improvements in analytical techniques coupled with detailed knowledge of the genes coding for the mucin proteins have provided exciting new insights into the role of the mucous layer and its relevance to gastrointestinal disease.
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Heterogeneity of airways mucus: variations in the amounts and glycoforms of the major oligomeric mucins MUC5AC and MUC5B.
TL;DR: The findings indicate that M UC5AC and MUC5B are the major oligomeric mucins and that airways mucus contains variable amounts of these glycoproteins, and can be altered significantly in diseased airways with, for instance, an increase in the low-charge form of the Muc5B mucin in CF and COPD mucus.
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Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator–dependent bicarbonate secretion
TL;DR: In a mouse model of CF (mice homozygous for the most common human CFTR mutation), intestinal mucus release was minimal when stimulated with either PGE2 or 5-HT in the presence or absence of HCO3-.
Journal ArticleDOI
Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure
Ashley G. Henderson,Camille Ehre,Brian Button,Lubna H. Abdullah,Liheng Cai,Margaret W. Leigh,Genevieve DeMaria,Hiro Matsui,Scott H. Donaldson,C. William Davis,John K. Sheehan,Richard C. Boucher,Mehmet Kesimer +12 more
TL;DR: It is shown that the concentration of mucin in CF sputum is low when measured by immunologically based techniques, and mass spectrometric analyses of CF mucins revealed mucin cleavage at antibody recognition sites, and mucin concentrations in CF secretions were higher than those in normal secretions.
Journal ArticleDOI
CFTR, Mucins, and Mucus Obstruction in Cystic Fibrosis
TL;DR: In this paper, a detailed examination of CFTR expression in organs and different cell types indicates that changes in CFTR expressions do not always correlate with the severity of CF disease or mucus accumulation.