|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. Full-size image|
The UNC CF Center 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 UNC CF Center 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 (Figure 17), steroids, macrolides, and inhaled osmolytes.