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Phenotypes Associated with This Genotype
Genotype
MGI:5430750
Allelic
Composition
Bmpr2tm1.1Enl/Bmpr2tm1.1Enl
Tg(Acvrl1-cre)L1Spo/0
Genetic
Background
involves: 129S4/SvJae * FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmpr2tm1.1Enl mutation (1 available); any Bmpr2 mutation (20 available)
Tg(Acvrl1-cre)L1Spo mutation (0 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• mutants with pulmonary hypertension exhibit an increased number of alpha-smooth muscle actin-positive (alphaSMA+) distal pulmonary arteries
• mutants with pulmonary hypertension exhibit an increase in medial wall thickness of alphaSMA+ small pulmonary arteries
• mutants exhibit a thickening of alphaSMA+ cell layers in small arteries of the pulmonary hypertension lungs and some arteries appear occluded, resembling the concentric vascular lesions in human pulmonary arterial hypertension
• 50% of mutant lungs show focal leukocyte infiltrations surrounding pulmonary vessels that are not seen in controls; infiltration is more frequent in mice with pulmonary hypertension than in mutants with lower right ventricular systolic pressure
• most of the infiltrating cells are CD68+ monocyte/macrophages
• ratio of right ventricle to left ventricle plus septum is higher in mutants with pulmonary hypertension than in controls, indicating right ventricle hypertrophy
• smooth muscle cells in distal pulmonary arteries exhibit increased proliferation index
• mutants exhibit a higher right ventricular systolic pressure than controls
• some mutants develop pulmonary hypertension (ventricular systolic pressure > 30 mm Hg)

cellular
• endothelial cells in distal pulmonary arteries exhibit increased proliferation index

homeostasis/metabolism
• 53% of pulmonary hypertension lungs exhibit in situ thrombosis, with lumens of some affected vessels partially or completely occluded by fibrin(ogen)+ thrombi

muscle
• smooth muscle cells in distal pulmonary arteries exhibit increased proliferation index

respiratory system
• 50% of mutant lungs show focal leukocyte infiltrations surrounding pulmonary vessels that are not seen in controls; infiltration is more frequent in mice with pulmonary hypertension than in mutants with lower right ventricular systolic pressure
• most of the infiltrating cells are CD68+ monocyte/macrophages
• endothelial cells in distal pulmonary arteries exhibit increased proliferation index

Mouse Models of Human Disease
OMIM ID Ref(s)
Pulmonary Hypertension, Primary, 1; PPH1 178600 J:158116


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
09/13/2016
MGI 6.05
The Jackson Laboratory