Phenotypes associated with this allele

• Allele Symbol: Tg(Tagln-rtTA)E1Jwst
• Allele Name: transgene insertion E1, James West
• Allele ID: MGI:3703755
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Tg(Tagln-rtTA)E1Jwst/0 Tg(tetO-BMPR2*T504)1Jwst/0	involves: FVB/N	MGI:3703798
cx2	Tg(Tagln-rtTA)E1Jwst/0 Tg(tetO-Bmpr2*R899X)#Jwst/0	involves: FVB/N	MGI:4838758

Genotype
MGI:3703798

cx1

• Allelic Composition: Tg(Tagln-rtTA)E1Jwst/0; Tg(tetO-BMPR2*T504)1Jwst/0
• Genetic Background: involves: FVB/N

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

cardiovascular system

abnormal vascular smooth muscle morphology ( J:98898 )

• medial thickness of muscular pulmonary arteries is increased in DOX-treated mice at 8 weeks

• there is increased muscularization of small pulmonary arteries at the level of alveolar ducts

abnormal heart ventricle morphology ( J:98898 )

• right ventricle/left ventricle (RV/LV) + septum weight is increased 30% in DOX-treated mutants at 8 weeks

increased heart right ventricle weight ( J:98898 )

• right ventricle relative weight is increased compared to controls at 8 weeks

increased right ventricle systolic pressure ( J:98898 )

• transgenic mice receive doxycycline (DOX) in food up to 8 weeks of age, when assays are performed; at 8 weeks of age, DOX-treated mutants show 2-fold higher right ventricular systolic pressure

hypertension ( J:98898 )

• DOX-treated mice show evidence of sustained pulmonary hypertension

muscle

abnormal vascular smooth muscle morphology ( J:98898 )

• medial thickness of muscular pulmonary arteries is increased in DOX-treated mice at 8 weeks

• there is increased muscularization of small pulmonary arteries at the level of alveolar ducts

hematopoietic system

increased hematocrit ( J:98898 )

• modest increase in hematocrit in 8 week old DOX-treated mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
primary pulmonary hypertension		DOID:14557	OMIM:178600 OMIM:265400 OMIM:615342 OMIM:615343 OMIM:615344	J:98898

Genotype
MGI:4838758

cx2

• Allelic Composition: Tg(Tagln-rtTA)E1Jwst/0; Tg(tetO-Bmpr2*R899X)#Jwst/0
• Genetic Background: involves: FVB/N

cardiovascular system

abnormal lung vasculature morphology ( J:164503 )

• doxycycline-treated mice exhibit pulmonary vascular pruning as determined by microangiography and decreased perfusion of pulmonary vasculature that worsens as right ventricular systolic pressure increases compared to in wild-type mice

• doxycycline-treated mice develop pulmonary vascular lesions with some vessels filled with well-organized layers of smooth muscle and other vessels filled with endothelial unlike in wild-type mice

• muscular lesions in doxycycline-treated mice are associated with macrophages, T cells, and CD133+ cells unlike in wild-type mice

• after 9 weeks of doxycycline treatment, mice exhibit small vessels filled with CD45+ cells unlike in wild-type mice

• however, pulmonary vasculature is present

heart right ventricle hypertrophy ( J:164503 )

• corresponding to increased right ventricular systolic pressure in doxycycline-treated mice

increased right ventricle systolic pressure ( J:164503 )

• in doxycycline-treated mice

growth/size/body

heart right ventricle hypertrophy ( J:164503 )

• corresponding to increased right ventricular systolic pressure in doxycycline-treated mice

increased body weight ( J:164503 )

• in doxycycline-treated mice

respiratory system

abnormal lung vasculature morphology ( J:164503 )

• doxycycline-treated mice exhibit pulmonary vascular pruning as determined by microangiography and decreased perfusion of pulmonary vasculature that worsens as right ventricular systolic pressure increases compared to in wild-type mice

• doxycycline-treated mice develop pulmonary vascular lesions with some vessels filled with well-organized layers of smooth muscle and other vessels filled with endothelial unlike in wild-type mice

• muscular lesions in doxycycline-treated mice are associated with macrophages, T cells, and CD133+ cells unlike in wild-type mice

• after 9 weeks of doxycycline treatment, mice exhibit small vessels filled with CD45+ cells unlike in wild-type mice

• however, pulmonary vasculature is present

muscle

heart right ventricle hypertrophy ( J:164503 )

• corresponding to increased right ventricular systolic pressure in doxycycline-treated mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
primary pulmonary hypertension		DOID:14557	OMIM:178600 OMIM:265400 OMIM:615342 OMIM:615343 OMIM:615344	J:164503