Phenotypes associated with this allele
mortality/aging
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• survival is reduced by about 70% at P3
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respiratory system
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• airway necrosis is increased compared to single Tg(Scgb1a1-Scnn1b)6608Bouc mice
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• airway inflammation levels are similar to single Tg(Scgb1a1-Scnn1b)6608Bouc mice
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• intraluminal mucus obstruction is increased compared to single Tg(Scgb1a1-Scnn1b)6608Bouc mice
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immune system
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• airway inflammation levels are similar to single Tg(Scgb1a1-Scnn1b)6608Bouc mice
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cellular
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• airway necrosis is increased compared to single Tg(Scgb1a1-Scnn1b)6608Bouc mice
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Scgb1a1-Scnn1b)6608Bouc mutation
(5 available)
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mortality/aging
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• Background Sensitivity: around 14% of mice die due to airway mucus obstruction, indicating reduced mortality on the C57BL/6 background compared to a mixed C3H and C57BL/6 or BALB/c congenic background
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respiratory system
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• Background Sensitivity: increase in the number of necrotic cells in the airway epithelium, however the level of necrosis is less on the C57BL/6 background compared to the BALB/c background in neonates
• extent of airway necrosis in 3 week old mice does not differ on the BALB/c or C57BL/6 background
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• extent of goblet cell metaplasia is similar on the C57BL/6 and the BALB/c backgrounds
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• increase in basal and amiloride-sensitive short-circuit current in neonatal tracheal tissue, indicating increased airway absorption of Na+
• cAMP-induced short-circuit current is increased in neonatal tracheal tissue
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• airway inflammation, with similar levels on the C57BL/6 or BALB/c backgrounds
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• extent of emphysema formation in 3 week old mice does not differ on the C57BL/6 or BALB/c backgrounds
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• Background Sensitivity: mucus obstruction is seen in the trachea but not in the intrapulmonary airways of neonates, with mucus content increased to a lesser extent on the C57BL/6 background compared to the BALB/c background
• however, extent of airway mucus obstruction in 3 week old mice does not differ on the C57BL/6 and the BALB/c backgrounds
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immune system
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• airway inflammation, with similar levels on the C57BL/6 or BALB/c backgrounds
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cellular
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• Background Sensitivity: increase in the number of necrotic cells in the airway epithelium, however the level of necrosis is less on the C57BL/6 background compared to the BALB/c background in neonates
• extent of airway necrosis in 3 week old mice does not differ on the BALB/c or C57BL/6 background
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Scgb1a1-Scnn1b)6608Bouc mutation
(5 available)
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mortality/aging
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• Background Sensitivity: 81% of mice die due to airway mucus obstruction, indicating increased mortality on the BALB/c background compared to a mixed C3H and C57BL/6 or C57BL/6 congenic background
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respiratory system
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• Background Sensitivity: the number of necrotic cells in the airway epithelium is increased, with a greater increase on the BALB/c background compared to the C57BL/6 background in neonates
• extent of airway necrosis in 3 week old mice does not differ on the BALB/c or C57BL/6 background
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• extent of goblet cell metaplasia is similar on the C57BL/6 and the BALB/c background
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• increase in basal and amiloride-sensitive short-circuit current in neonatal tracheal tissue, indicating increased airway absorption of Na+
• cAMP-induced short-circuit current is increased in neonatal tracheal tissue
• Background Sensitivity: bumetanide-sensitive and CFTRinh-172-sensitive short-circuit current is decreased in tracheal tissues of neonates on the BALB/c background compared to on the C57BL/6 background, indicating that basal chloride secretion is lower on the BALB/c background
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• airway inflammation, with similar levels on the C57BL/6 or BALB/c backgrounds
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• extent of emphysema formation in 3 week old mice does not differ on the C57BL/6 or BALB/c background
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• Background Sensitivity: mucus obstruction is seen in the trachea but not in the intrapulmonary airways of neonates, with mucus content increased to a greater extent on the BALB/c background compared to the C57BL/6 background
• extent of airway mucous obstruction in 3 week old mice does not differ on the BALB/c or C57BL/6 background
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immune system
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• airway inflammation, with similar levels on the C57BL/6 or BALB/c backgrounds
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cellular
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• Background Sensitivity: the number of necrotic cells in the airway epithelium is increased, with a greater increase on the BALB/c background compared to the C57BL/6 background in neonates
• extent of airway necrosis in 3 week old mice does not differ on the BALB/c or C57BL/6 background
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Scgb1a1-Scnn1b)6608Bouc mutation
(5 available)
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mortality/aging
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• although transgenic animals are born at the expected Mendelian ratios, 50% of transgenic mice of both sexes die from the first days of postnatal life through 4 weeks of age
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respiratory system
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• airway epithelia of adult transgenic mice exhibit goblet cell metaplasia
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• increased airway absorption of Na+ is demonstrated by elevation of basal and amiloride-sensitive short-circuit currents (Isc) across freshly excised adult and neonatal tracheal tissue of transgenic mice over those of nontransgenic littermates; in contrast, both forskolin and UTP fail to increase chloride channel activation in amiloride pretreated transgenic tracheae above wild-type levels
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• histopathologic examination of the lungs reveals evidence of chronic bronchitis, the lumina of conducting airways populated with macrophages and neutrophils; however, the submucosa contain few inflammatory cells
• bronchoalveolar lavage (BAL) fluid and lung homogenates of adult, but not neonatal, transgenic mice contain significantly higher levels of macrophage inflammatory protein 2 (MIP-2) and KC than do those of nontransgenic littermates
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• lung histology of all transgenic mice is normal at birth, but those that die early exhibit severe obstruction of small and large airways by mucus plaques and plugs; those euthanized after 4 weeks have less severe mucus obstruction with dilation of airspaces distal to blockages
• airway mucus of transgenic mice is significantly more concentrated than that of nontransgenic littermates; electron and light microscopy reveal adhesion of mucus to airway epithelia and reduction of periciliary liquid (PCL) height in tracheae and bronchi of transgenic mice
• the rate of clearance of a fluorescent dye demonstrates slower in vitro mucus transport in the lower airways of transgenic mice than of nontransgenic littermates, and whereas intratracheally instilled Haemophilus influenzae or Pseudomonas aeruginosa were almost completely cleared within 3 days from the lungs of wild-type mice, transgenic mice retained a significant bacterial burden
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• early deaths of transgenic mice result from asphyxia due to severe airway obstruction, evidenced by frequent observation of intercostal and subdiaphragmatic retractions
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immune system
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• histopathologic examination of the lungs reveals evidence of chronic bronchitis, the lumina of conducting airways populated with macrophages and neutrophils; however, the submucosa contain few inflammatory cells
• bronchoalveolar lavage (BAL) fluid and lung homogenates of adult, but not neonatal, transgenic mice contain significantly higher levels of macrophage inflammatory protein 2 (MIP-2) and KC than do those of nontransgenic littermates
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Analysis Tools