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Emerging Therapeutic Approaches for Cystic Fibrosis

Review ARTICLE Provisionally accepted The full-text will be published soon. Notify me

Front. Pharmacol. | doi: 10.3389/fphar.2018.01475

Animal models in the pathophysiology of Cystic Fibrosis

  • 1Physiology & Biophysics, Dalhousie University, Canada

Our understanding of the multiorgan pathology of cystic fibrosis (CF) has improved impressively during the last decades, but we still lack a full comprehension of the disease progression. Animal models have greatly contributed to the elucidation of specific mechanisms involved in CF pathophysiology and the development of new therapies. Soon after the cloning of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in 1989, the first mouse model was generated and this model has dominated in vivo CF research ever since. Nonetheless, the failure of murine models to mirror human disease severity in the pancreas and lung has led to the generation of larger animal models such as pigs and ferrets. The following review presents and discusses data from the current animal models used in CF research.

Keywords: cystic fibrosis, CF mice, animals in CF, CF ferrets, CF pigs, CF rats

Received: 27 Sep 2018; Accepted: 03 Dec 2018.

Edited by:

Miquéias Lopes-Pacheco, Universidade de Lisboa, Portugal

Reviewed by:

Rainer Schreiber, University of Regensburg, Germany
Susan E. Birket, University of Alabama at Birmingham, United States  

Copyright: © 2018 Semaniakou, Croll and Chappe. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Dr. Valerie Chappe, Dalhousie University, Physiology & Biophysics, Halifax, B3H 4R2, Nova Scotia, Canada,