Effect of Ap<sub>4</sub>A, UTP and Salbutamol on Mucociliary Clearance in a Mouse Model of Cystic Fibrosis (<i>in Situ</i>)

• 作者: Eugen J. Verspohl Frank Begrow
• 来源期刊: 药理与制药(英文)
• 学科: 医学
• 关键词: CYSTIC Fibrosis Mucociliary Clearance in Situ Ap4A UTP
• 年，卷（期）: 2013,（2）
• 页码范围: 176-181
• 页数: 6页
• 分类号: R73

摘要

Cystic fibrosis is a life-threatening, wide spread genetic disease diagnosed in 1 to 3000 livebirths of the Caucasian population. Here a mouse model for this disease is described and optimized using the CFTR-channel selective inhibitor CFTR(inh 172). The target parameter was mucociliary clearance measured using microdialysis of the transported fluorescent dye rhodamine in the mouse trachea in situ. The impact of Ap4A (diadenosine tetraphosphate) as a potential drug was investigated. Its inhalation was effective at low concentrations;established compounds such as Salbutamol and UTP increased mucociliary clearance as well. Our data show a functioning model of cystic fibrosis and the effectiveness of the newly tested Ap4A.