Book chapter
Cystic Fibrosis and Defective Airway Innate Immunity
Antimicrobial Peptides and Innate Immunity, pp.275-306
Progress in Inflammation Research, Springer Basel
12/10/2012
DOI: 10.1007/978-3-0348-0541-4_11
Abstract
Cystic fibrosis is a common autosomal recessive disease caused by mutations in the CFTR gene that encodes an anion channel expressed in epithelia and other cell types. While the disease affects multiple organ systems, it is progressive pulmonary disease, characterized by airway infection and inflammation, that is life limiting. The origins of the lung disease associated with loss of CFTR function are complex and likely multifactorial. Current research is defining how loss of CFTR anion channel activity alters the volume and composition of respiratory secretions and thereby impacts host defenses. Here we review the current understanding of the defect in innate immunity that characterizes the airway disease in cystic fibrosis. Advances in cystic fibrosis basic science research and the development of new animal models of disease are shedding new light on the causes of lung disease and may lead to new, more targeted therapies.
Details
- Title: Subtitle
- Cystic Fibrosis and Defective Airway Innate Immunity
- Creators
- Jennifer A. Bartlett - University of IowaPaul B. McCray - University of Iowa
- Resource Type
- Book chapter
- Publication Details
- Antimicrobial Peptides and Innate Immunity, pp.275-306
- Series
- Progress in Inflammation Research
- DOI
- 10.1007/978-3-0348-0541-4_11
- Publisher
- Springer Basel; Basel
- Language
- English
- Date published
- 12/10/2012
- Academic Unit
- Microbiology and Immunology; Pulmonary Medicine; Stead Family Department of Pediatrics; Internal Medicine
- Record Identifier
- 9984297427302771
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