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Methods: Spdef knockout mice were crossed to Scnn1b-Tg mice and phenotypes of the progenies were evaluated. Specifically, Muc5ac and Muc5b levels were measured in unfractionated BAL by agarose western blots, goblet cell metaplasia was evaluated by AB-PAS staining in histological sections, Muc5b expression determined by immunohistochemistry, and airway inflammation measured by BAL differential cell counts.

Results: Both neonatal and adult Spdef-deficient Scnn1b-Tg mice displayed significant mucus plugging with high levels of Muc5b in BALs as compared to wild-type littermates. Muc5ac in BALs was inhibited by loss of Spdef in adult but not neonatal Scnn1b-Tg mice. Consistent with the known function of Spdef to limit inflammatory responses, a gene dose-dependent increase in neutrophils was observed in Spdef-deficient Scnn1b-Tg mice as compared to Spdef-sufficient Scnn1b-Tg mice during …