Cystic fibrosis is a multi-system lethal genetic disorder. Mutations in the gene that encodes
for the cystic fibrosis transmembrane conductance regulator (CFTR) are the basis of this
disorder. Currently, a variety of CF mouse models have been developed for in-depth studies.
They provide significant insight into the pathophysiology; however, these models do not
always mimic the human disease. In this review we will summarize the available models and
highlight the characteristics that have made them important tools for advancing our …