Journal article
Effects of airway surface liquid pH on host defense in cystic fibrosis
The international journal of biochemistry & cell biology, Vol.52, pp.124-129
07/2014
DOI: 10.1016/j.biocel.2014.02.009
PMCID: PMC4404156
PMID: 24560894
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.
Details
- Title: Subtitle
- Effects of airway surface liquid pH on host defense in cystic fibrosis
- Creators
- Abigail R Berkebile - Department of Microbiology, Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USAPaul B McCray - Department of Microbiology, Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA
- Resource Type
- Journal article
- Publication Details
- The international journal of biochemistry & cell biology, Vol.52, pp.124-129
- DOI
- 10.1016/j.biocel.2014.02.009
- PMID
- 24560894
- PMCID
- PMC4404156
- NLM abbreviation
- Int J Biochem Cell Biol
- ISSN
- 1357-2725
- eISSN
- 1878-5875
- Publisher
- Elsevier BV
- Grant note
- name: NIH, award: P01 HL-51670, P01 HL-091842; name: National Science Foundation Graduate Research Fellowship, award: 1048957
- Language
- English
- Date published
- 07/2014
- Academic Unit
- Microbiology and Immunology; Pulmonary Medicine; Stead Family Department of Pediatrics; Internal Medicine
- Record Identifier
- 9984093369702771
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