title: Effects of airway surface liquid pH on host defense in cystic fibrosis.
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.
authors.affiliation: Department of Microbiology, Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA.
authors.forename: Abigail R
authors.initials: AR
authors.lastname: Berkebile
authors.affiliation: Department of Microbiology, Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA; Department of Pediatrics, Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA. Electronic address: paul-mccray@uiowa.edu.
authors.forename: Paul B
authors.initials: PB
authors.lastname: McCray
mesh_terms: D000818:Animals; D002478:Cells, Cultured; D003550:Cystic Fibrosis / Q000276:immunology* / Q000378:metabolism*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000276:immunology / Q000378:metabolism; D006801:Humans; D006863:Hydrogen-Ion Concentration; D009079:Mucociliary Clearance; D020545:Respiratory Mucosa / Q000276:immunology* / Q000378:metabolism* / Q000382:microbiology
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: ASL; Airway surface liquid; Antimicrobials; Cystic fibrosis