All Phenotypes Bmpr2<+> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Bmpr2⁺
wild type
MGI:2434614

Summary

9 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
ht1	Bmpr2^tm1Mmue/Bmpr2⁺	B6.129S1-Bmpr2^tm1Mmue	MGI:5827840
ht2	Bmpr2^tm1.1Pley/Bmpr2⁺	B6.Cg-Bmpr2^tm1.1Pley	MGI:5568733
ht3	Bmpr2^tm1Kmi/Bmpr2⁺	involves: 129S4/SvJae	MGI:3526244
ht4	Bmpr2^tm1Kmi/Bmpr2⁺	involves: 129S4/SvJae * C57BL/6	MGI:5430760
ht5	Bmpr2^tm1Kmi/Bmpr2⁺	involves: 129S4/SvJae * C57BL/6J	MGI:5438770
ht6	Bmpr2^tm1.2Nwm/Bmpr2⁺	Not Specified	MGI:8255484
cx7	Bmpr2^tm1Mmue/Bmpr2⁺ Smad1^tm1Rjle/Smad1⁺	involves: 129S1/SvImJ * 129S6/SvEvTac * C57BL/6	MGI:5827848
cx8	Bmpr2^tm1Kmi/Bmpr2⁺ Btg2^tm1Spo/Btg2^tm1Spo	involves: 129S4/SvJae * C57BL/6	MGI:3526247
cx9	Bmpr2^tm1Kmi/Bmpr2⁺ Ark2c^Gt(P9-3F)Sor/Ark2c⁺ Tg(Hlxb9-GFP)1Tmj/0	involves: 129S4/SvJae * C57BL/6 * CBA	MGI:5516589

Allelic Composition	Bmpr2^tm1Mmue/Bmpr2⁺
Genetic Background	B6.129S1-Bmpr2^tm1Mmue

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmpr2^tm1Mmue mutation (0 available); any Bmpr2 mutation (46 available)

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

cardiovascular system

abnormal pulmonary artery morphology ( J:224752 )

• 6 month old mice exhibit enhanced muscularization of peripheral pulmonary arteries in the lung

increased right ventricle systolic pressure ( J:224752 )

• 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

pulmonary hypertension ( J:224752 )

• 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

increased vascular permeability ( J:224752 )

• 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

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

Allelic Composition	Bmpr2^tm1.1Pley/Bmpr2⁺
Genetic Background	B6.Cg-Bmpr2^tm1.1Pley

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmpr2^tm1.1Pley mutation (0 available); any Bmpr2 mutation (46 available)

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

cardiovascular system

cardiovascular system phenotype ( J:209027 )

N	• mice do not spontaneously develop pulmonary arterial hypertension

Allelic Composition	Bmpr2^tm1Kmi/Bmpr2⁺
Genetic Background	involves: 129S4/SvJae

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmpr2^tm1Kmi mutation (0 available); any Bmpr2 mutation (46 available)

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

skeleton

abnormal sternocostal joint morphology ( J:95124 )

• eight ribs, instead of nine, were attached to the sternum

vertebral transformation ( J:95124 )

• exhibited alleviated vertebral defects compared to homozygous Acvr2b mutants

• transformation of the 23rd vertebra to the 16th thoracic vertebra (as seen in homozygous Acvr2b mutants) was incomplete

• vertebra 29 of most mutants was transformed to the first sacral vertebra instead to the 6th lumbar vertebra (as seen in homozygous Acvr2b mutants)

Allelic Composition	Bmpr2^tm1Kmi/Bmpr2⁺
Genetic Background	involves: 129S4/SvJae * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmpr2^tm1Kmi mutation (0 available); any Bmpr2 mutation (46 available)

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

cardiovascular system

abnormal pulmonary artery morphology ( J:98219 )

• heterozygotes exhibit an increase in wall thickness of muscularized pulmonary arteries

• heterozygotes exposed to hypoxia exhibit impaired muscularization of small pulmonary arteries in both the distal intra-acinar vessels and in proximal preacinar vessels, with 25% less increase in wall thickness compared to controls

abnormal lung vasculature morphology ( J:98219 )

• the number of alveoli (and associated capillaries called alveolar-capillary units) per millimeter squared is 40% greater in heterozygotes

• heterozygotes exposed to prolonged hypoxia exhibit impaired pulmonary vascular remodeling, with a smaller increase in pulmonary artery pressure, greater right ventricular hypertrophy, decrease in the number of alveolar-capillary units per millimeter squared, and decreased wall thickness (muscularization) of small pulmonary arteries compared to controls

increased pulmonary vascular resistance ( J:98219 )

• increase in mean total pulmonary vascular resistance and in incremental pulmonary resistance

• however total systemic vascular resistance is not different

increased pulmonary artery pressure ( J:98219 )

• increase in mean pulmonary arterial pressure

• however, mean systemic arterial pressure is not different from controls

• heterozygotes exposed to prolonged hypoxia show a smaller increase in pulmonary artery pressure than control mice

pulmonary hypertension ( J:98219 )

• mutants develop mild pulmonary hypertension

respiratory system

abnormal lung vasculature morphology ( J:98219 )

• the number of alveoli (and associated capillaries called alveolar-capillary units) per millimeter squared is 40% greater in heterozygotes

abnormal pulmonary alveolus morphology ( J:98219 )

• the number of alveoli (and associated capillaries called alveolar-capillary units) per millimeter squared is 40% greater in heterozygotes, decreasing the vessels-to-alveoli ratio

Allelic Composition	Bmpr2^tm1Kmi/Bmpr2⁺
Genetic Background	involves: 129S4/SvJae * C57BL/6J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmpr2^tm1Kmi mutation (0 available); any Bmpr2 mutation (46 available)

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

cardiovascular system

abnormal lung vasculature morphology ( J:117167 )

• mutants with adenovirus-mediated pulmonary overexpression of 5-lipoxygenase to induce inflammation in the lungs exhibit a greater number of muscularized vessels than in the lungs from wild-type mice

pulmonary hypertension ( J:117167 )

• mutants with adenovirus-mediated pulmonary overexpression of 5-lipoxygenase to induce inflammation in the lungs exhibit increased right ventricular systolic pressure compared to wild-type mice with the same treatment, indicating increased sensitivity to stress-induced pulmonary hypertension

• however, mutants exhibit normal right ventricular systolic pressure and do not develop pulmonary hypertension spontaneously under unstressed conditions

increased vasoconstriction ( J:117167 )

• mutants with adenovirus-mediated pulmonary overexpression of 5-lipoxygenase to induce inflammation in the lungs exhibit enhanced pulmonary vasoconstriction

immune system

abnormal leukocyte physiology ( J:117167 )

• minor increase in adhesion of leukocytes to the vessel wall in the lungs

mortality/aging

perinatal lethality, incomplete penetrance ( J:117167 )

• about 20% fewer than the expected number of heterozygous mice were detected, indicating loss in early development

• most mice that do not survive, die in utero, although some die in the immediate postnatal stage

prenatal lethality, incomplete penetrance ( J:117167 )

muscle

increased vasoconstriction ( J:117167 )

• mutants with adenovirus-mediated pulmonary overexpression of 5-lipoxygenase to induce inflammation in the lungs exhibit enhanced pulmonary vasoconstriction

respiratory system

abnormal lung vasculature morphology ( J:117167 )

hematopoietic system

abnormal leukocyte physiology ( J:117167 )

• minor increase in adhesion of leukocytes to the vessel wall in the lungs

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:117167

Allelic Composition	Bmpr2^tm1.2Nwm/Bmpr2⁺
Genetic Background	Not Specified

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmpr2^tm1.2Nwm mutation (0 available); any Bmpr2 mutation (46 available)

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

cardiovascular system

cardiovascular system phenotype ( J:367200 )

N	• no changes in right ventricular systolic pressure and no right ventricular hypertrophy (RVH) • no changes in pulmonary artery wall thickness, with or without 4PBA treatment

respiratory system

abnormal pulmonary alveolar duct morphology ( J:367200 )

• reduced alveolar duct artery muscularization after 4PBA treatment: increase in nonmuscularized and decrease in partially muscularized vessels

Allelic Composition	Bmpr2^tm1Mmue/Bmpr2⁺ Smad1^tm1Rjle/Smad1⁺
Genetic Background	involves: 129S1/SvImJ * 129S6/SvEvTac * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmpr2^tm1Mmue mutation (0 available); any Bmpr2 mutation (46 available)
Smad1^tm1Rjle mutation (0 available); any Smad1 mutation (34 available)

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

cardiovascular system

heart right ventricle hypertrophy ( J:224752 )

• right ventricular hypertrophy by 6 months of age

increased right ventricle systolic pressure ( J:224752 )

• by 6 months of age, mice develop more severe elevations in right ventricular systolic pressure than single Bmpr2 heterozygotes

growth/size/body

heart right ventricle hypertrophy ( J:224752 )

• right ventricular hypertrophy by 6 months of age

muscle

heart right ventricle hypertrophy ( J:224752 )

• right ventricular hypertrophy by 6 months of age

Allelic Composition	Bmpr2^tm1Kmi/Bmpr2⁺ Btg2^tm1Spo/Btg2^tm1Spo
Genetic Background	involves: 129S4/SvJae * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bmpr2^tm1Kmi mutation (0 available); any Bmpr2 mutation (46 available)
Btg2^tm1Spo mutation (0 available); any Btg2 mutation (17 available)

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

skeleton

vertebral transformation ( J:95124 )

• mutants exhibited similar vertebral abnormalities as single homozygous Btg2 mutants, with no differences in the severity of phenotype

Allelic Composition	Bmpr2^tm1Kmi/Bmpr2⁺ Ark2c^Gt(P9-3F)Sor/Ark2c⁺ Tg(Hlxb9-GFP)1Tmj/0
Genetic Background	involves: 129S4/SvJae * C57BL/6 * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ark2c^Gt(P9-3F)Sor mutation (0 available); any Ark2c mutation (11 available)
Bmpr2^tm1Kmi mutation (0 available); any Bmpr2 mutation (46 available)
Tg(Hlxb9-GFP)1Tmj mutation (3 available)

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

mortality/aging

prenatal lethality, incomplete penetrance ( J:198828 )

• fewer than expected mice are born

• however, all born mice survive to adulthood

nervous system

abnormal motor neuron innervation pattern ( J:198828 )

• some mice exhibit innervation defects in the dorsal forelimb

behavior/neurological

decreased grip strength ( J:198828 )

• some mice exhibit reduced hang time from a cage lid compared with wild-type mice

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