CF (cystic fibrosis) is a severe autosomal recessive disease most common in Northwest European populations. Underlying mutations in the CFTR (CF transmembrane conductance regulator) gene cause deregulation of ion transport and subsequent dehydration of the airway surface liquid, producing a viscous mucus layer on the airway surface of CF patients. This layer is readily colonized by bacteria such as Pseudomonas aeruginosa. Owing to the resulting environment and treatment strategies, the bacteria acquire genetic modifications such as antibiotic resistance, biofilm formation, antimicrobial peptide resistance and pro-inflammatory lipid A structures. Lipid A is a component of the lipopolysaccharide cell wall present on bacteria and is recognized by TLR4 (Toll-like receptor 4). Its detection elicits a pro-inflammatory response that is heightened over time due to the addition of fatty acids to the lipid A structure. Eradication of bacteria from the lungs of CF patients becomes increasingly difficult and eventually leads to mortality. In the present review, we describe the role of lipid A as a virulent factor of Ps. aeruginosa; however, it appears that further work is needed to investigate the role of CFTR in the innate immune response and in modifying the pathogen–host interaction.
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Conference Article|
July 22 2009
Role of CFTR, Pseudomonas aeruginosa and Toll-like receptors in cystic fibrosis lung inflammation
Paul J. Buchanan;
Paul J. Buchanan
1
*School of Medicine, Dentistry and Biomedical Science, Centre for Infection and Immunity, Respiratory Research, Queens University Belfast, Belfast BT12 6BN, U.K.
1To whom correspondence should be addressed (email pbuchanan03@qub.ac.uk).
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Robert K. Ernst;
Robert K. Ernst
†Department of Microbial Pathogenesis, University of Maryland Baltimore, Baltimore, MD 21201, U.S.A.
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J. Stuart Elborn;
J. Stuart Elborn
*School of Medicine, Dentistry and Biomedical Science, Centre for Infection and Immunity, Respiratory Research, Queens University Belfast, Belfast BT12 6BN, U.K.
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Bettina Schock
Bettina Schock
*School of Medicine, Dentistry and Biomedical Science, Centre for Infection and Immunity, Respiratory Research, Queens University Belfast, Belfast BT12 6BN, U.K.
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Publisher: Portland Press Ltd
Received:
March 04 2009
Online ISSN: 1470-8752
Print ISSN: 0300-5127
© The Authors Journal compilation © 2009 Biochemical Society
2009
Biochem Soc Trans (2009) 37 (4): 863–867.
Article history
Received:
March 04 2009
Citation
Paul J. Buchanan, Robert K. Ernst, J. Stuart Elborn, Bettina Schock; Role of CFTR, Pseudomonas aeruginosa and Toll-like receptors in cystic fibrosis lung inflammation. Biochem Soc Trans 1 August 2009; 37 (4): 863–867. doi: https://doi.org/10.1042/BST0370863
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