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Cystic Fibrosis and Defective Airway Innate Immunity

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Antimicrobial Peptides and Innate Immunity

Part of the book series: Progress in Inflammation Research ((PIR))

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.

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Acknowledgements

This work was supported by P01 HL-51670, P01 HL-091842, and the Roy J. Carver Charitable Trust. We thank Erin Burnight, Jeydith Gutierrez, and Sateesh Krishnamurthy for critically reviewing the manuscript. We thank Tom Moninger and David Meyerholz for assistance in preparing photomicrographs.

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  1. Department of Pediatrics, University of Iowa, Iowa City, IA, 52242, USA

    Jennifer A. Bartlett

  2. 240F EMRB, Department of Pediatrics, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, IA, 52242, USA

    Paul B. McCray Jr.

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  1. Jennifer A. Bartlett
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  2. Paul B. McCray Jr.
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Correspondence to Paul B. McCray Jr. .

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  1. , Department of Pulmonology, Leiden University Medical Center, Albinusdreef 2, Leiden, 2300 RC, Netherlands

    Pieter S. Hiemstra

  2. Ctr for Infection & Immun. Amsterdam, Dept. Medical Microbiology, University of Amsterdam, Meibergdreef 15, Amsterdam, 1105 AZ, Netherlands

    Sebastian A. J. Zaat

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Bartlett, J.A., McCray, P.B. (2013). Cystic Fibrosis and Defective Airway Innate Immunity. In: Hiemstra, P., Zaat, S. (eds) Antimicrobial Peptides and Innate Immunity. Progress in Inflammation Research. Springer, Basel. https://doi.org/10.1007/978-3-0348-0541-4_11

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