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Published ahead of print on August 3, 2006, doi:10.1165/rcmb.2006-0184TR

Am. J. Respir. Cell Mol. Biol., Volume 36, Number 1, January 2007, 1-7

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Submitted on May 26, 2006
Revised on August 2, 2006

Cystic Fibrosis Mouse Models

Claudine Guilbault1, Zienab Saeed1, Gregory P Downey2, and Danuta Radzioch1*

1 McGill Centre for the Study of Host Resistance, McGill University Health Center Research Institute, Montreal, Quebec, Canada, 2 Division of Respirology, Department of Medicine, University of Toronto and Toronto General Hospital Research Institute of the University Health Network, Toronto, Ontario, Canada

* To whom correspondence should be addressed. E-mail: danuta.radzioch{at}muhc.mcgill.ca.

Animal models of cystic fibrosis (CF) are powerful tools that enable the study of the mechanisms and complexities of human disease. Murine models have several intrinsic advantages compared to other animal models including lower cost, maintenance, and rapid reproduction rate. Mice can be easily genetically manipulated by making transgenic or knockout mice, or by backcrossing to well-defined inbred strains in a reasonably short period of time. However, anatomical and immunological differences between mice and humans mean that murine models have inherent limitations that must be considered when interpreting the results obtained from experimental models and applying these to the pathogenesis of CF disease in humans. This review will focus on the different CF mouse models available that represent diverse phenotypes observed in humans with CF and that can help researchers elucidate the diverse functions of the CFTR protein.




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