Immunopathology of Airway Surface Liquid Dehydration Disease

Table 1

Mouse models of single ion-channel defect.

S. No.	Model name	Strain background	Transgene/mutation	Spontaneous onset of lung disease	Airway mucus obstruction	Airway mucous cell metaplasia	Spontaneous airway bacterial infection	Chronic airway inflammation	Mortality	Reference
1	Cftr^tm 1UNC	C57BL/6	Mutation (exon 10)	No	Absent	Present only upon bacterial challenge	Absent	Neutrophilic infiltration at day 30	Yes	(Snouwaert et al., [15])
2	Cftr^tm 1HGU	MF1	Mutation (exon 10)	No	Absent	Present only upon bacterial challenge	Absent	Absent	No	(Dorin et al., [17])
3	Cftr^tm 1CAM	C57BL/6	Mutation (exon 10)	No	Absent	Absent	Absent	Absent	Yes	(Ratcliff et al., [19])
4	Cftr^tm 1BAY		Mutation (exon 3)	No	Absent	Absent	Absent	Absent	Yes	(O’Neal et al., [20])
5	Cftr^tm 3BAY	129/Sv	Mutation (exon 2)	No	Absent	Absent	Absent	Absent	Yes	(Hasty et al., [21])
6	Cftr^tm 2CAM	C57BL/6	Mutation (ΔF508)	No	Absent	Absent	Absent	Absent	Yes	(Colledge et al., [23])
7	Cftr^tm 1EUR	FVB	Mutation (ΔF508)	No	Absent	Absent	Absent	Absent	No	(van Doorninck et al., [25])
8	Cftr^tm 1KTH		Mutation (ΔF508)	No	Absent	Absent	Absent	Absent	Yes	(Zeiher et al., [24])
9	Cftr^tm 1HSC	129/SV	Mutation (exon 1)	No	Absent	Absent	Absent	Absent	Yes	(Rozmahel et al., [22])
10	Cftr^G5 51D	CD1/129	Mutation (G551D)	No	Absent	Absent	Absent	Absent	Yes	(Delaney et al., [26])
11	Cftr^tm 2HGU		Mutation (G480C)	No	Absent	Absent	Absent	Absent	No	(Dickinson et al., [27])
12	Scnn1b-Tg+	C3H : C57	Transgene (Scnn1b)	Yes	Yes	Yes	Postnatal	Yes	Yes	(Mall et al., [36])
13	Cftr^tm 2UTH	C57BL/6	Mutation (R117H)	No	Absent	Absent	Absent	Absent	No	(van Heeckeren et al., [28])
14	Cftr^tm 3UTH	C57BL/6	Mutation (Y122X)	No	Absent	Absent	Absent	Absent	Yes	(van Heeckeren et al., [28])