Phenotypes Associated with This Genotype

Genotype
MGI:4838758

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

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

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