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• W1985870591 abstract "Cystic fibrosis (CF) lung disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene and is characterized by a perpetuated feedback loop of bacterial infection and inflammation. Both intrinsic (CFTR-dependent) and extrinsic (CFTR-independent) mechanisms contribute to the inflammatory phenotype of CF lung disease. Innate immune cells, initially recruited to combat bacterial pathogens, are acting in a dysregulated and non-resolving fashion in CF airways and cause harm to the host by releasing proteases and oxidants. Targeting harmful immune pathways, while preserving protective ones, remains the challenge for the future. This review highlights current concepts of innate immune dysregulation in CF lung disease." @default.
• W1985870591 created "2016-06-24" @default.
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• W1985870591 date "2014-07-01" @default.
• W1985870591 modified "2023-10-05" @default.
• W1985870591 title "Current concepts of immune dysregulation in cystic fibrosis" @default.
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• W1985870591 doi "https://doi.org/10.1016/j.biocel.2014.01.017" @default.
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