The innate immune system in cystic fibrosis lung disease
نویسندگان
چکیده
منابع مشابه
The innate immune system in cystic fibrosis lung disease.
The Journal of Clinical Investigation | February 1999 | Volume 103 | Number 3 303 The story of cystic fibrosis (CF) research over the last decade has provided important lessons about the power and limitations of positional cloning of disease genes, the understanding of pathogenesis, and the development of new therapies. Studies in the 1980s identified defects in the chloride conductance propert...
متن کاملInnate immunity in cystic fibrosis lung disease.
Chronic lung disease determines the morbidity and mortality of cystic fibrosis (CF) patients. The pulmonary immune response in CF is characterized by an early and non-resolving activation of the innate immune system, which is dysregulated at several levels. Here we provide a comprehensive overview of innate immunity in CF lung disease, involving (i) epithelial dysfunction, (ii) pathogen sensing...
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Pulmonary infection is the dominant clinical feature of cystic fibrosis (CF), but the basis for this susceptibility remains incompletely understood. One hypothesis is that CF airway surface liquid (ASL) is abnormal and interferes with neutrophil function. To study this possibility, we developed an in vitro system in which we collected ASL from primary cultures of normal and CF airway epithelial...
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A 32-year-old man was admitted in the Emergency Department with acute dyspnea secondary to spontaneous pneumothorax. He had history of spontaneous pneumothorax 2 years ago.He was active cigarette smoker (10 pack/year). The family history was unremarkable for lung diseases. Additionally, he had no systemic complaints.The lung HRCT revealed multiple bizarre-shaped cysts distributed in both lungs ...
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Since the isolation of the gene responsible for cystic fibrosis in 1989 we have witnessed rapid progress in our understanding of this common genetic disorder. Progressively more detailed knowledge of the disease causing mutations, the gene expression patterns, the protein structure, and its function as an ion channel has allowed the development of reliable screening programmes, the creation of ...
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ژورنال
عنوان ژورنال: Journal of Clinical Investigation
سال: 1999
ISSN: 0021-9738
DOI: 10.1172/jci6277