Open AccessCystic fibrosis and innate immunity: how chloride channel mutations provoke lung disease
Author(s) -
Döring Gerd,
Gulbins Erich
Publication year - 2009
Publication title -
cellular microbiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.542
H-Index - 138
eISSN - 1462-5822
pISSN - 1462-5814
DOI - 10.1111/j.1462-5822.2008.01271.x
Subject(s) - innate immune system , cystic fibrosis , biology , cystic fibrosis transmembrane conductance regulator , immunology , immunity , chloride channel , mucociliary clearance , disease , immune system , lung , fibrosis , antimicrobial peptides , microbiology and biotechnology , genetics , pathology , medicine , antimicrobial
Summary Innate immunity is essential for prevention of infection in vertebrates and plants and dysfunction of single components of innate immunity may provoke severe disease. Here we describe how mutations in the cystic fibrosis transmembrane conductance regulator gene dysregulate a variety of components of the innate immune system in individuals suffering from the hereditary disease cystic fibrosis. In the airways of these individuals, functions of the mucociliary clearance system, cationic antimicrobial (poly)peptides and neutrophils and macrophages are impaired and inflammatory signal transduction pathways exaggerated. Consequently, chronic airway colonization with opportunistic bacterial pathogens develops and leads to life‐threatening lung disease.