Mouse Genome Informatics
cx1
    Tg(Tagln-rtTA)E1Jwst/0
Tg(tetO-BMPR2*T504)1Jwst/0

involves: FVB/N
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
cardiovascular system
• 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
• right ventricle/left ventricle (RV/LV) + septum weight is increased 30% in DOX-treated mutants at 8 weeks
• right ventricle relative weight is increased compared to controls at 8 weeks
• 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
• DOX-treated mice show evidence of sustained pulmonary hypertension

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

Mouse Models of Human Disease
OMIM IDRef(s)
Pulmonary Hypertension, Primary, 1; PPH1 178600 J:98898


Mouse Genome Informatics
cx2
    Tg(Tagln-rtTA)E1Jwst/0
Tg(tetO-Bmpr2*R899X)#Jwst/0

involves: FVB/N
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
cardiovascular system
• 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
• corresponding to increased right ventricular systolic pressure in doxycycline-treated mice
• in doxycycline-treated mice

growth/size/body
• in doxycycline-treated mice

respiratory system
• 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

Mouse Models of Human Disease
OMIM IDRef(s)
Pulmonary Hypertension, Primary, 1; PPH1 178600 J:164503