Animal models of chronic lung infection with Pseudomonas aeruginosa: useful tools for cystic fibrosis studies.

摘要: Cystic fibrosis (CF) is caused by a defect in the transmembrane conductance regulator (CFTR) protein that functions as chloride channel. Dysfunction of CFTR results salty sweat, pancreatic insufficiency, intestinal obstruction, male infertility and severe pulmonary disease. In most patients with CF life expectancy limited due to progressive loss functional lung tissue. Early persistent neutrophylic inflammation can be demonstrated airways. The cause this inflammation, role morbidity different CF-specific bacteria, mostly Pseudomonas aeruginosa, are not well understood. lack an appropriate animal model multi-organ pathology having characteristics human form has hampered our understanding pathobiology chronic infections disease for many years. This review summarizes main focuses on several available models have been frequently used research. A better infection particularly P. pathophysiology pathogenesis necessitates understand CF, develop improve treatment.