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- W2157380634 endingPage "412" @default.
- W2157380634 startingPage "389" @default.
- W2157380634 abstract "Summary Cystic fibrosis (CF) is caused by a defect in the transmembrane conductance regulator (CFTR) protein that functions as a chloride channel. Dysfunction of the CFTR protein results in salty sweat, pancreatic insufficiency, intestinal obstruction, male infertility and severe pulmonary disease. In most patients with CF life expectancy is limited due to a progressive loss of functional lung tissue. Early in life a persistent neutrophylic inflammation can be demonstrated in the airways. The cause of this inflammation, the role of CFTR and the cause of lung morbidity by different CF-specific bacteria, mostly Pseudomonas aeruginosa, are not well understood. The lack of an appropriate animal model with multi-organ pathology having the characteristics of the human form of CF has hampered our understanding of the pathobiology and chronic lung infections of the disease for many years. This review summarizes the main characteristics of CF and focuses on several available animal models that have been frequently used in CF research. A better understanding of the chronic lung infection caused particularly by P. aeruginosa, the pathophysiology of lung inflammation and the pathogenesis of lung disease necessitates animal models to understand CF, and to develop and improve treatment." @default.
- W2157380634 created "2016-06-24" @default.
- W2157380634 creator A5058001103 @default.
- W2157380634 creator A5070467243 @default.
- W2157380634 date "2008-10-01" @default.
- W2157380634 modified "2023-09-27" @default.
- W2157380634 title "Animal models of chronic lung infection with <i>Pseudomonas aeruginosa</i>: useful tools for cystic fibrosis studies" @default.
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