Abstract
The susceptibility of cystic fibrosis patients to bacterial pathogens is associated with deficient airway antimicrobial peptide activity, and airway-surface-liquid dehydration with decreased transport velocity and hypersecretion of mucus. Susceptibility to Pseudomonas aeruginosa infection has been linked to the role of the cystic fibrosis transmembrane conductance regulator protein as a receptor for P. aeruginosa. Binding of P. aeruginosa coordinates lung clearance as part of innate immunity. The function of CFTR in innate immunity to P. aeruginosa infection is multifactorial, with one key component being a specific ligand-receptor interaction between the protein and the microbe.
Publication types
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Research Support, U.S. Gov't, P.H.S.
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Review
MeSH terms
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Animals
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Apoptosis
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Cystic Fibrosis / complications*
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Cystic Fibrosis / genetics*
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Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
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Disease Susceptibility
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Humans
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Immunity, Innate
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Lung / microbiology*
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Models, Biological
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Mutation
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Proto-Oncogene Proteins / metabolism
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Proto-Oncogene Proteins / pharmacokinetics
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Proto-Oncogene Proteins c-fyn
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Pseudomonas Infections / complications*
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Pseudomonas aeruginosa*
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Respiratory Tract Infections / genetics
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Respiratory Tract Infections / microbiology*
Substances
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CFTR protein, human
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Proto-Oncogene Proteins
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Cystic Fibrosis Transmembrane Conductance Regulator
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FYN protein, human
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Proto-Oncogene Proteins c-fyn