title: Airway epithelial control of Pseudomonas aeruginosa infection in cystic fibrosis.
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.
authors.affiliation: Channing Laboratory, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, USA.
authors.forename: Victoria L
authors.initials: VL
authors.lastname: Campodónico
authors.forename: Mihaela
authors.initials: M
authors.lastname: Gadjeva
authors.forename: Catherine
authors.initials: C
authors.lastname: Paradis-Bleau
authors.forename: Ahmet
authors.initials: A
authors.lastname: Uluer
authors.forename: Gerald B
authors.initials: GB
authors.lastname: Pier
mesh_terms: D000818:Animals; D001422:Bacterial Adhesion; D001980:Bronchi / Q000382:microbiology*; D003550:Cystic Fibrosis / Q000276:immunology / Q000382:microbiology* / Q000517:prevention & control / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000276:immunology / Q000378:metabolism*; D004847:Epithelial Cells / Q000382:microbiology* / Q000648:ultrastructure; D006193:Haemophilus influenzae / Q000472:pathogenicity; D054884:Host-Pathogen Interactions; D006801:Humans; D008168:Lung / Q000382:microbiology / Q000473:pathology; D051379:Mice; D011552:Pseudomonas Infections / Q000276:immunology / Q000382:microbiology* / Q000517:prevention & control / Q000628:therapy; D011550:Pseudomonas aeruginosa / Q000472:pathogenicity / Q000502:physiology; D013211:Staphylococcus aureus / Q000472:pathogenicity; D037521:Virulence Factors / Q000502:physiology
chemical_list: D037521:Virulence Factors; D019005:Cystic Fibrosis Transmembrane Conductance Regulator