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The Marsico Lung Institute has numerous animal models of lung diseases, including the first 鈥淐F mouse鈥� (黑料网^tm 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 尾ENaC transgenic mouse. 尾ENaC 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. 尾ENaC mice have been used for multiple drug studies, including ENaC blockers (Fig. 17), steroids, macrolides, and inhaled osmolytes.

Figure 17. Whole histologic sections of 尾ENaC and wild type (WT) mice, stained with AB-PAS. Note mucus-obstructed airway in 尾ENac-Tg mice (upper left). Mucus obstruction is cleared with treatment with ENaC blocker (upper right). WT controls shown below.