These Scnn1b-transgenic mice overexpress the mouse nonvoltage-gated 1 beta, Scnn1b, under the direction of the rat secretoglobin, family 1A, member 1 (uteroglobin; Clara cell secretory protein) promoter. Approximately 40-60% of hemizygous mice die between birth and 4 weeks of age due to airway obstruction asphyxia. While the donating investigator reports that most hemizygous transgenic mice (80-90%) on the congenic C57BL/6N background (Stock No. 030949) survive past postnatal day 14, hemizygous mice on the congenic C57BL/6J (Stock No. 006438), B6;C3H mixed (Stock No. 005315), and B6C3Fe hybrid (Stock No. 006176) backgrounds at The Jackson Laboratory exhibit an approximately 40% survival rate to weaning age. Histological analysis reveals that mucus accumulation, plaques and plugs in airways occur postnatally. Basal and amiloride-sensitive short-circuit currents in tracheal tissue are increased. Airway surface liquid (ASL) volume, mucus transport and clearance are reduced. Bronchial lavage and histological analysis shows mutant mice exhibit characteristics of cystic fibrosis lung disease including chronic bronchitis, airway inflammation, airway lumen infiltration of macrophage and neutrophils, and goblet cell metaplasia. These Scnn1b-transgenic mice may be useful in studies of cystic fibrosis, and are available on different genetic backgrounds such as B6;C3H mixed (Stock No. 005315), B6C3Fe hybrid (Stock No. 006176), C57BL6-congenic (Stock No. 006438), and congenic C57BL/6N (Stock No. 030949).
Importation of this model was supported by The Boomer Esiason Foundation.
