Effects of airway surface liquid pH on host defense in cystic fibrosis |
| |
| Affiliation: | 2. Galapagos Medicinal Chemistry, Romainville, France;3. Galapagos Medicinal Chemistry, Mechelen, Belgium;1. Department of Medicine, Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, University of Minnesota, United States;2. Department of Medicine, Division of Gastroenterology, Hepatology, and Nutrition, University of Minnesota, United States;1. Marsico Lung Institute, University of North Carolina, Chapel Hill, USA;2. Department of Mathematics, University of North Carolina, Chapel Hill, USA;3. Department of Applied Physical Sciences, University of North Carolina, Chapel Hill, USA;4. Department of Biomedical Engineering, University of North Carolina, Chapel Hill, USA;5. National Health and Environmental Effects Research Laboratory, United States Environmental Protection Agency, University of North Carolina, Chapel Hill, USA;6. Center for Environmental Medicine Asthma and Lung Biology, University of North Carolina, Chapel Hill, USA;7. Telethon Kids Institute, University of Western Australia, Perth, Australia;8. Division of Pediatrics, University of Western Australia, Perth, Australia;9. Princess Margaret Hospital for Children, Perth, Australia;10. Division of Pediatric Pulmonology, University of North Carolina at Chapel Hill, Chapel Hill, NC, 27599, USA;11. Department of Physics and Astrophysics, University of North Carolina at Chapel Hill, Chapel Hill, NC, 27599, USA |
| |
| Abstract: | Cystic fibrosis is a lethal genetic disorder characterized by viscous mucus and bacterial colonization of the airways. Airway surface liquid represents a first line of pulmonary defense. Studies in humans and animal models of cystic fibrosis indicate that the pH of airway surface liquid is reduced in the absence of cystic fibrosis transmembrane conductance regulator function. Many aspects of the innate host defense system of the airways are pH sensitive, including antimicrobial peptide/protein activity, the rheological properties of secreted mucins, mucociliary clearance, and the activity of proteases. This review will focus on how changes in airway surface liquid pH may contribute to the host defense defect in cystic fibrosis soon after birth. Understanding how changes in pH impact mucosal immunity may lead to new therapies that can modify the airway surface liquid environment, improve airway defenses, and alter the disease course.This article is part of a Directed Issue entitled: Cystic Fibrosis: From o-mics to cell biology, physiology, and therapeutic advances. |
| |
| Keywords: | Cystic fibrosis Antimicrobials Airway surface liquid ASL |
| 本文献已被 ScienceDirect 等数据库收录！ |
|
