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Progress in understanding mucus abnormalities in cystic fibrosis airways

Journal article
Authors J. J. Wine
Gunnar C. Hansson
P. Konig
N. S. Joo
Anna Ermund
M. Pieper
Published in Journal of Cystic Fibrosis
Volume 17
Issue 2
Pages S35-S39
ISSN 1569-1993
Publication year 2018
Published at Institute of Biomedicine, Department of Medical Biochemistry and Cell Biology
Pages S35-S39
Language en
Links dx.doi.org/10.1016/j.jcf.2017.09.00...
Keywords Cystic fibrosis, Mucus, Mucociliary clearance, HCO3-, Hypertonic saline, Submucosal glands, mucociliary clearance, surface dehydration, hypertonic saline, submucosal glands, epithelial-cells, mucin, secretion, lung, pathogenesis, bicarbonate, Respiratory System, ates of america, v105, p15064, ates of america, v114, p6842
Subject categories Respiratory Medicine and Allergy

Abstract

Normal airways below the carina maintain an essentially sterile environment via a multi-pronged innate defence system that includes mucus clearance via mucociliary clearance and cough, multiple antimicrobials and cellular components including macrophages and neutrophils. In cystic fibrosis (CF), loss of CFTR function compromises these defences, and with present standard of care virtually all people with CF eventually develop mucus accumulation, plugging and chronic infections. This review focuses on how mucus is affected by CFTR loss. (C) 2017 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

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