CFTR-dependent susceptibility of the cystic fibrosis-host to Pseudomonas aeruginosa

摘要: Cystic fibrosis is the most common autosomal recessive disorder in western countries. The disease characterized by recurrent and chronic infections of lung particular with Pseudomonas aeruginosa, Staphylococcus aureus, Burkholderia cepacia, Haemophilus influenzae. Albeit intensive research last years, molecular mechanisms causing high susceptibility cystic patients to bacterial are still unknown. Animal models provided important novel information on pathophysiology mimicked many pathological findings humans, for instance inflammation increased infection susceptibility. These animal were recently employed identify several proteins lipids that critically involved fibrosis. Thus, studies identified death receptors, caveolin proteins, membrane rafts, alterations ceramide metabolism an accumulation lungs be susceptibility, inflammation, reduced mucociliary clearance