Phenotypes associated with this allele

• Allele Symbol: Tg(tetO-CTGF)#Swu
• Allele Name: transgene insertion, Shu Wu
• Allele ID: MGI:5473718
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Tg(Scgb1a1-rtTA)1Jaw/0 Tg(tetO-CTGF)#Swu/0	involves: 129 * C57BL/6 * C57BL/6J	MGI:5473719
cx2	Tg(SFTPC-rtTA)2Jaw/0 Tg(tetO-CTGF)#Swu/0	involves: 129 * C57BL/6 * C57BL/6J	MGI:5473834

Genotype
MGI:5473719

cx1

• Allelic Composition: Tg(Scgb1a1-rtTA)1Jaw/0; Tg(tetO-CTGF)#Swu/0
• Genetic Background: involves: 129 * C57BL/6 * C57BL/6J

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

respiratory system

abnormal lung vasculature morphology ( J:171488 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit decreased capillary formation in alveolar septa

impaired lung alveolus development ( J:171488 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit disrupted alveolarization, showing a 27% and 31% decrease in the mean alveolar airspace area and mean cord length, respectively

• pups nursing on dams administered doxycycline from P1 to P14 exhibit disorganized elastic fiber deposition on alveolar surfaces and in septa

abnormal pulmonary interalveolar septum morphology ( J:171488 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit a 34% decrease in secondary septa

• marker analysis indicates that pups nursing on dams administered doxycycline from P1 to P14 exhibit increased myofibroblast differentiation in alveolar septa

• pups nursing on dams administered doxycycline from P1 to P14 exhibit a 48% increase in the proliferating cell index in alveolar septa

• pups nursing on dams administered doxycycline from P1 to P14 exhibit decreased capillary formation in alveolar septa

pulmonary fibrosis ( J:171488 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit thickening in the peribronchial/peribronchiolar and perivascular regions of the lungs and increased collagen deposition in these regions, indicating fibrosis

cardiovascular system

abnormal lung vasculature morphology ( J:171488 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit decreased capillary formation in alveolar septa

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
lower respiratory tract disease		DOID:0050161		J:171488

Genotype
MGI:5473834

cx2

• Allelic Composition: Tg(SFTPC-rtTA)2Jaw/0; Tg(tetO-CTGF)#Swu/0
• Genetic Background: involves: 129 * C57BL/6 * C57BL/6J

respiratory system

abnormal lung vasculature morphology ( J:171151 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit a 60% decrease of vWF+ intraacinous vessels in lungs at P14, indicating inhibition of pulmonary vascular development

• pups nursing on dams administered doxycycline from P1 to P14 exhibit a 70% increase in muscularization of pulmonary vessels, a 2-fold increase in medial wall thickness of vessels, and increased vessels with proliferating cells in the lungs, indicating pulmonary vascular remodeling

lung inflammation ( J:171151 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit increased inflammatory cell infiltration in alveolar air spaces and perivascular regions of lungs, with a 9-fold increase in macrophage infiltration

• pups nursing on dams administered doxycycline from P1 to P14 exhibit an increase in macrophage and neutrophil counts and the presence of numerous multinucleated giant cells in bronchoalveolar lavage (BAL) fluids

impaired lung alveolus development ( J:171151 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit larger and simplified alveoli with fewer secondary septa compared to controls at P7 and P14

• pups nursing on dams administered doxycycline from P1 to P14 show a 30% and 50% increase in mean linear intercept in lungs at P7 and P14, respectively and a 60% reduction in radial alveolar count at P14

abnormal pulmonary interalveolar septum morphology ( J:171151 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit simplified alveoli with fewer secondary septa compared to controls at P7 and P14

cardiovascular system

abnormal lung vasculature morphology ( J:171151 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit a 60% decrease of vWF+ intraacinous vessels in lungs at P14, indicating inhibition of pulmonary vascular development

• pups nursing on dams administered doxycycline from P1 to P14 exhibit a 70% increase in muscularization of pulmonary vessels, a 2-fold increase in medial wall thickness of vessels, and increased vessels with proliferating cells in the lungs, indicating pulmonary vascular remodeling

heart right ventricle hypertrophy ( J:171151 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit an increase in the ratio of right ventricle over left ventricle plus septum compared to controls, indicating hypertrophic right ventricles

increased right ventricle systolic pressure ( J:171151 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit a higher right ventricular systolic pressure compared to controls

pulmonary hypertension ( J:171151 )

• pups nursing on dams administered doxycycline from P1 to P14 develop pulmonary hypertension as indicated by increased right ventricle systolic pressure and right ventricular hypertrophy

immune system

lung inflammation ( J:171151 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit increased inflammatory cell infiltration in alveolar air spaces and perivascular regions of lungs, with a 9-fold increase in macrophage infiltration

• pups nursing on dams administered doxycycline from P1 to P14 exhibit an increase in macrophage and neutrophil counts and the presence of numerous multinucleated giant cells in bronchoalveolar lavage (BAL) fluids

muscle

heart right ventricle hypertrophy ( J:171151 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit an increase in the ratio of right ventricle over left ventricle plus septum compared to controls, indicating hypertrophic right ventricles

growth/size/body

heart right ventricle hypertrophy ( J:171151 )

• pups nursing on dams administered doxycycline from P1 to P14 exhibit an increase in the ratio of right ventricle over left ventricle plus septum compared to controls, indicating hypertrophic right ventricles

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
lower respiratory tract disease		DOID:0050161		J:171151