Animal Models of Lung Disease
The Marsico Lung Institute has numerous animal models of lung diseases, including the first “CF mouse” (UNCtm null). Current gene-targeted models include MUC1, 4, and 16 –/– mice, P2Y2-R –/– and P2Y4-R –/– mice, and pannexin –/– mice.
In addition, the Institute has the muco-obstructive βENaC transgenic mouse. βENaC mice have been crossed onto multiple strains and differing levels of transgene expression have developed. The mice exhibit a phenotype of airway surface liquid volume depletion, mucus hyperconcentration and adhesion, inflammation, early bacterial infection, airway re-modeling, and emphysema. βENaC mice have been used for multiple drug studies, including ENaC blockers (Fig. 17), steroids, macrolides, and inhaled osmolytes.
Figure 17. Whole histologic sections of βENaC and wild type (WT) mice, stained with AB-PAS. Note mucus-obstructed airway in βENac-Tg mice (upper left). Mucus obstruction is cleared with treatment with ENaC blocker (upper right). WT controls shown below.