Effect of Ap₄A, UTP and Salbutamol on Mucociliary Clearance in a Mouse Model of Cystic Fibrosis (in Situ)

Frank Begrow, Eugen J. Verspohl

Department of Pharmacology, Institute of Medicinal Chemistry, University of Muenster, Muenster, Germany.

DOI: 10.4236/pp.2013.42025                

Abstract

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.

Keywords

Cystic Fibrosis; Mucociliary Clearance in Situ; Ap₄A; UTP

Conflicts of Interest

The authors declare no conflicts of interest.

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