Review Article

Immunopathology of Airway Surface Liquid Dehydration Disease

Table 2

Various genetic modifications in the Scnn1b-Tg+ mouse model.

GenotypeDescriptionMacrophage infiltrationNeutrophil infiltrationEosinophil infiltrationLymphocyte infiltrationMucous cell metaplasiaAirway mucus obstructionDistal airspace enlargementReference

Tnfα-/--Scnn1b-Tg+Global deletion of TnfαNo significant differenceNo significant differenceNo significant differenceNo significant differenceNo significant differenceNo significant differenceNo significant difference(Livraghi et al., [65])
Tnfr1-/--Scnn1b-Tg+Global deletion of Tnfr1No significant differenceNo significant differenceNo significant differenceNo significant differenceNo significant differenceNo significant differenceNo significant difference(Livraghi et al., [65])
Il4rα-/--Scnn1b-Tg+Global deletion of Il4rαNo significant differenceNo significant differenceSignificantly reduced at PND 10 and 5 weeks of ageNo significant differenceSignificantly reduced at PND 10No significant differenceNo significant difference(Livraghi et al., [65])
Myd88-/-Scnn1b-Tg+Global deletion of Myd88Significantly higher at PND 10; no significant difference at other observed time-pointsSignificantly reducedNo significant differenceLymphoid hyperplasia significantly increased at 8 weeks of ageSignificantly reduced at PND 5-7, but not at other time-pointsSignificantly reduced at PND 5-7, but not at any other observed time-pointNot reported(Livraghi-Butrico et al., [38])
Ne-/--Scnn1b-Tg+Global deletion of neutrophil elastaseNo significant differenceSignificantly reducedNo significant differenceNo significant differenceSignificantly reducedNo significant differenceSignificantly reduced(Gehrig et al., [57])
Il1r-/--Scnn1b-Tg+Global deletion of Il1rNo significant differenceSignificantly reducedNo significant differenceNo significant differenceNot reportedSignificantly reducedSignificantly reduced(Fritzsching et al., [97])
DTA+-Scnn1b-Tg+Partial deficiency of macrophagesNo significant difference in total number of macrophage infiltration, significantly reduced in total percentageSignificantly increasedNo significant differenceIncreased occurrence of lymphoid aggregates in adult mice; significant infiltration in nonemaciated phenotypeSignificantly reduced in emaciated phenotypeSignificantly reduced in emaciated phenotypeNo significant difference(Saini et al., [51, 53])
Muc5b-/--Scnn1b-Tg+Global deletion of Muc45bNo significant differenceNo significant differenceNot reportedIncreased lymphoid aggregates, but no significant difference in BALF lymphocytesNot reportedSignificantly reducedNo significant difference(Livraghi-Butrico et al., [108])
Muc5ac-/--Scnn1b-Tg+Global deletion of Muc5acNo significant differenceNo significant differenceNot reportedNo significant difference in incidence of lymphoid aggregatesNot reportedSignificantly reducedNot reported(Livraghi-Butrico et al., [108])
Spdef-/--Scnn1b-Tg+Global deletion of SpdefNo significant differenceSignificantly increased in neonatesNo significant differenceNo significant differenceNot reportedNo significant differenceNot reported(Chen et al., [109])