Allele Symbol Allele Name Allele ID |
Bmpr2tm1Mmue targeted mutation 1, Matthias Mueller MGI:5776726 |
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Summary |
3 genotypes
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• 6 month old mice exhibit enhanced muscularization of peripheral pulmonary arteries in the lung
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• mice exhibit normal right ventricular systolic pressure at 3 months of age but develop elevated right ventricular systolic pressure by 6 months of age
• however, mice do not exhibit right ventricular hypertrophy and show no differences in left ventricular end diastolic pressure or cardiac output at 6 months
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• mice exhibit pulmonary arterial hypertension by 6 months of age
• treatment of 6 month old mice with daily intraperitoneal injection of BMP9 for 4 weeks reverses all indices of pulmonary arterial hypertension, returning right ventricular systolic pressure to normal and reversing the enhanced pulmonary arterial muscularization
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• monolayers of pulmonary endothelial cells in vitro show increased permeability at baseline and following LPS stimulation
• mice injected with LPS develop increased pulmonary vascular leak compared to wild-type mice
• treatment with BMP9 prevents vascular leak to a similar degree as in wild-type mice
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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:224752 |
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• right ventricular hypertrophy by 6 months of age
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• by 6 months of age, mice develop more severe elevations in right ventricular systolic pressure than single Bmpr2 heterozygotes
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• right ventricular hypertrophy by 6 months of age
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• right ventricular hypertrophy by 6 months of age
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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 11/12/2024 MGI 6.24 |
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