Abstract
Defective expression or function of the cystic fibrosis transmembrane conductance regulator (CFTR) underlies the hypersusceptibility of cystic fibrosis (CF) patients to chronic airway infections, particularly with Pseudomonas aeruginosa. CFTR is involved in the specific recognition of P. aeruginosa, thereby contributing to effective innate immunity and proper hydration of the airway surface layer (ASL). In CF, the airway epithelium fails to initiate an appropriate innate immune response, allowing the microbe to bind to mucus plugs that are then not properly cleared because of the dehydrated ASL. Recent studies have identified numerous CFTR-dependent factors that are recruited to the epithelial plasma membrane in response to infection and that are needed for bacterial clearance, a process that is defective in CF patients hypersusceptible to infection with this organism.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Animals
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Bacterial Adhesion
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Bronchi / microbiology*
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Cystic Fibrosis / immunology
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Cystic Fibrosis / microbiology*
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Cystic Fibrosis / prevention & control
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Cystic Fibrosis / therapy
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Cystic Fibrosis Transmembrane Conductance Regulator / genetics
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Cystic Fibrosis Transmembrane Conductance Regulator / immunology
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Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
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Epithelial Cells / microbiology*
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Epithelial Cells / ultrastructure
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Haemophilus influenzae / pathogenicity
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Host-Pathogen Interactions
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Humans
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Lung / microbiology
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Lung / pathology
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Mice
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Pseudomonas Infections / immunology
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Pseudomonas Infections / microbiology*
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Pseudomonas Infections / prevention & control
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Pseudomonas Infections / therapy
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Pseudomonas aeruginosa / pathogenicity
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Pseudomonas aeruginosa / physiology
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Staphylococcus aureus / pathogenicity
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Virulence Factors / physiology
Substances
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Virulence Factors
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Cystic Fibrosis Transmembrane Conductance Regulator