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M. T. Clunes and R. C. Boucher, “Front-Runners for Pharmacotherapeutic Correction of the Airway Ion Transport Defect in Cystic Fibrosis,” Current Opinion in Pharmacology, Vol. 8, No. 3, 2008, pp. 292-299. doi:10.1016/j.coph.2008.04.006

has been cited by the following article:

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

  AUTHORS: Frank Begrow, Eugen J. Verspohl

  KEYWORDS: Cystic Fibrosis; Mucociliary Clearance in Situ; Ap4A; UTP

  JOURNAL NAME: Pharmacology & Pharmacy, Vol.4 No.2, April 15, 2013

  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.