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Allele Symbol Allele Name Allele ID |
Tg(Pdgfb-icre/ERT2,-EGFP)1Frut transgene insertion 1, Marcus Fruttiger MGI:3793852 |
| Summary |
17 genotypes |
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|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• in lungs of E17.5 embryos after tamoxifen exposure of the mothers
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• reduced branching in placenta and yolk sac in E12.5 embryos 3 days after tamoxifen exposure of the mothers
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• in retina of P6.5 pups after tamoxifen exposure at P0, P1 and P2
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|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• following treatment with tamoxifen, retinal vascular density is reduced compared to in wild-type retina
• however, endothelial junctional assemblies are normal
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• excessive in the retina following tamoxifen treatment
|
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• following treatment with tamoxifen, retinal vascular density is reduced compared to in wild-type retina
• however, endothelial junctional assemblies are normal
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• excessive in the retina following tamoxifen treatment
|
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|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• embryos exposed to tamoxifen
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• delayed vascularization of the superficial retinal vascular plexus at 5-12 days of age
• vessels are unstable
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• endothelial cell proliferation is increased
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• reduced vascular density and decreased number of branch points in the superficial retinal vascular plexus
• capillary network massively reduced in the deep capillary plexus
• capillary orientation in the superficial plexus relative to the deep plexus is disrupted
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• delayed vascularization of the superficial retinal vascular plexus at 5-12 days of age
• vessels are unstable
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• endothelial cell proliferation is increased
|
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• reduced vascular density and decreased number of branch points in the superficial retinal vascular plexus
• capillary network massively reduced in the deep capillary plexus
• capillary orientation in the superficial plexus relative to the deep plexus is disrupted
|
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• mice treated with tamoxifen at P1-4 show reduced vascular branching in retinal vessels at P5
• mice treated with tamoxifen at P1-4 show fewer distal sprouts with filopodia and fewer number of filopodia in the retinal vasculature
• distal sprouts have fewer side connections in the retinal vasculature of mice treated with tamoxifen at P1-4
• the radial expansion of the vascular plexus is reduced in mice treated with tamoxifen at P1-4
• vessel regression in the retina is increased in tamoxifen treated mice
|
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• endothelial cell proliferation is reduced
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• mice treated with tamoxifen at P1-4 show reduced vascular branching in retinal vessels at P5
• mice treated with tamoxifen at P1-4 show fewer distal sprouts with filopodia and fewer number of filopodia in the retinal vasculature
• distal sprouts have fewer side connections in the retinal vasculature of mice treated with tamoxifen at P1-4
• the radial expansion of the vascular plexus is reduced in mice treated with tamoxifen at P1-4
• vessel regression in the retina is increased in tamoxifen treated mice
|
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• with postnatal tamoxifen treatment from P1-P3, pups are generally smaller than tamoxifen-treated control littermates
|
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• perpendicular sprouting and neovascularization of the deeper retina are strongly compromised with tamoxifen treatment between P5 and P9
|
| N |
• stability of blood vessels is not affected in mutants with tamoxifen treatment at P1; stability of established vessels in the superficial capillary plexus is not affected with tamoxifen administration between P5 and P9
|
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• postnatal tamoxifen treatment from P1-P3 results in decreased coverage of retinal arteries by vascular smooth muscle cells; however pericyte coverage of capillary beds shows no change
|
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• perpendicular sprouting and neovascularization of the deeper retina are strongly compromised with tamoxifen treatment between P5 and P9
|
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• postnatal tamoxifen treatment from P1-P3 to delete Jag1 in endothelial cells results in significant decreases in numbers of filopodia and filopodia-extending endothelial tip cells
|
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• postnatal tamoxifen treatment from P1-P3 results in strong inhibition of angiogenesis in the retina resulting in reduced branching and delayed extension toward the periphery
|
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|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• slower growth of horizontal blood vessels in retina at age P7 after tamoxifen administration from age P1-P4
|
|
• increased blood vessel leakage in retina at age P7 after tamoxifen administration from age P1-P4
|
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• slower growth of horizontal blood vessels in retina at age P7 after tamoxifen administration from age P1-P4
• increased blood vessel leakage at age P7 after tamoxifen administration from age P1-P4
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| exudative vitreoretinopathy | DOID:0050535 |
OMIM:PS133780 |
J:328283 | |
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• slower growth of horizontal blood vessels in retina at age P3 during tamoxifen administration from age P1-P4
• slower growth of horizontal blood vessels in retina at age P9 after tamoxifen administration from age P1-P4
• delayed growth of vertical blood vessels in superficial retina vascular plexus at age P9 after tamoxifen administration from age P1-P
• lack of vertical secondary and tertiary vessels in retina at age P9 after tamoxifen administration from age P1-P4
• defects of vascular growth into deeper layers of retina at age P9 after tamoxifen administration from age P1-P4
• defects in periphery of superficial retina vascular plexus at age P13 after tamoxifen administration from age P6
• fewer vessels in retina OPL and minimal vessels in retina IPL at age P13 after tamoxifen administration from age P6
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• enlarged superficial vessels in retina at age P9 after tamoxifen administration from age P1-P4
• enlarged blood vessels in brain at age P9 after tamoxifen administration from age P1-P4
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• in eyes after tamoxifen administration from age P1-P4
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• extensive blood leakage in brain at age P9 after tamoxifen administration from age P1-P4
|
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• slower growth of horizontal blood vessels at age P3 during tamoxifen administration from age P1-P4
• slower growth of horizontal blood vessels and enlarged superficial vessels at age P9 after tamoxifen administration from age P1-P4
• delayed growth of vertical blood vessels in superficial retina vascular plexus at age P9 after tamoxifen administration from age P1-P4
• lack of vertical secondary and tertiary vessels at age P9 after tamoxifen administration from age P1-P4
• defects of vascular growth into deeper layers at age P9 after tamoxifen administration from age P1-P4
• defects in periphery of superficial retina vascular plexus at age P13 after tamoxifen administration from age P6
• fewer vessels in OPL and minimal vessels in IPL at age P13 after tamoxifen administration from age P6
• increased blood vessel leakage at age P9 after tamoxifen administration from age P1-P4
|
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• slower regression after tamoxifen administration from age P1-P4
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• after tamoxifen administration from age P1-P4
|
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• most mice die by age P9 after tamoxifen administration from age P1-P4
• mice die by age P13-P14 after tamoxifen administration from age P6
|
|
• extensive blood leakage in brain at age P9 after tamoxifen administration from age P1-P4
|
|
• after tamoxifen administration from age P1-P4
|
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• after tamoxifen administration from age P1-P4
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| exudative vitreoretinopathy | DOID:0050535 |
OMIM:PS133780 |
J:328283 | |
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• all mice treated with tamoxifen at birth form cerebral cavernous malformations in the brain by 2 months which progress in severity over time
• the lesions in mice treated with tamoxifen at birth show dilated, thin-walled caverns surrounded by hemosiderin deposits, inflammation, and fibrosis
|
|
• mice treated with tamoxifen at birth first show vessel dilation and failure of plexus coalescence in the vascular network at P7, and over time the vascular dilations resolve into caverns and appear as mature retinal cerebral cavernous malformation by P21
• cerebral cavernous malformation lesions in tamoxifen treated mice arise from the superficial vascular plexus and remain superficial until later in disease progression
• mice treated with tamoxifen at P7 develop lesions in the periphery of the retina by P21; these lesions show a similar morphology as those from mice treated with tamoxifen at birth, however severity and size are smaller
• mice treated with tamoxifen at P21 do not develop retinal lesions
|
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• mice treated with tamoxifen at birth exhibit vascular leak in the brain
|
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• mice treated with tamoxifen at P1 exhibit retinal endothelial hypersprouting
|
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• mice treated with tamoxifen at birth develop simple, dilated telangiectasias that progress to large multi-chambered caverns over time
|
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• presence of lesions in mice treated with tamoxifen at birth decrease the survival rate
|
|
• mice treated with tamoxifen at birth develop simple, dilated telangiectasias that progress to large multi-chambered caverns over time
|
|
• all mice treated with tamoxifen at birth form cerebral cavernous malformations in the brain by 2 months which progress in severity over time
• the lesions in mice treated with tamoxifen at birth show dilated, thin-walled caverns surrounded by hemosiderin deposits, inflammation, and fibrosis
|
|
• mice treated with tamoxifen at birth develop mature cerebral cavernous malformation in the retina that recapitulate retinal angiomas by P21
|
|
• mice treated with tamoxifen at birth first show vessel dilation and failure of plexus coalescence in the vascular network at P7, and over time the vascular dilations resolve into caverns and appear as mature retinal cerebral cavernous malformation by P21
• cerebral cavernous malformation lesions in tamoxifen treated mice arise from the superficial vascular plexus and remain superficial until later in disease progression
• mice treated with tamoxifen at P7 develop lesions in the periphery of the retina by P21; these lesions show a similar morphology as those from mice treated with tamoxifen at birth, however severity and size are smaller
• mice treated with tamoxifen at P21 do not develop retinal lesions
|
|
• mice treated with tamoxifen at birth develop mature cerebral cavernous malformation in the retina that recapitulate retinal angiomas by P21
|
|
• cavernous malformation lesions form in the retinas of mice treated with tamoxifen at birth
• retinal lesions in mice treated with tamoxifen at birth arise in the veins located both most superior and inferior within the retina
|
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• reduced proliferation rate after tamoxifen administration and 7 days after hind-limb ischemia (HLI) induction
|
|
• reduced intercapillary anastomosis in calf muscles after tamoxifen administration and 28 days after hind-limb ischemia (HLI) induction
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• impaired hind-limb ischemia (HLI)-induced revascularization after tamoxifen administration and 4 weeks after HLI-induction
• lower vascular density after tamoxifen administration and 7 days after hind-limb ischemia (HLI) induction
|
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• in calf muscles after tamoxifen administration and 28 days after hind-limb ischemia (HLI) induction
• same muscle damage as wildtype in calf muscles after tamoxifen administration and 1 day after hind-limb ischemia (HLI) induction
|
|
• in calf muscles after tamoxifen administration and 28 days after hind-limb ischemia (HLI) induction
|
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• impaired polarization toward M2-like phenotype in calf muscles after tamoxifen administration and 3 days after hind-limb ischemia (HLI) induction
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• impaired transition from M1-like to M2-like phenotype in calf muscles after tamoxifen administration and 3 days after hind-limb ischemia (HLI) induction
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• lower lactate levels in conditioned medium of calf muscle endothelial cells isolated after tamoxifen administration and hind-limb ischemia (HLI) induction
• virtually no increase in lactate levels in calf muscles after tamoxifen administration and 12h after hind-limb ischemia (HLI) induction
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• after tamoxifen administration and 12h after hind-limb ischemia (HLI) induction
|
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• extensive and widespread in calf muscles after tamoxifen administration and 3 days after hind-limb ischemia (HLI) induction
|
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• reduced glycolytic flux in calf muscle endothelial cells 10 days after tamoxifen administration
• lower extracellular acidification rate (ECAR) flux in calf muscle endothelial cells 10 days after tamoxifen administration
|
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• lower extracellular acidification rate (ECAR) flux in calf muscle endothelial cells 10 days after tamoxifen administration
|
|
• reduced glycolytic flux in calf muscle endothelial cells 10 days after tamoxifen administration
• lower extracellular acidification rate (ECAR) flux in calf muscle endothelial cells 10 days after tamoxifen administration
|
|
• impaired polarization toward M2-like phenotype in calf muscles after tamoxifen administration and 3 days after hind-limb ischemia (HLI) induction
|
|
• impaired transition from M1-like to M2-like phenotype in calf muscles after tamoxifen administration and 3 days after hind-limb ischemia (HLI) induction
|
| N |
• normal apoptosis rate of calf muscle endothelial cells after tamoxifen administration and 7 days after hind-limb ischemia (HLI) induction
|
|
• higher incidence of necrotic toes after tamoxifen administration and 4 weeks after hind-limb ischemia (HLI) induction
|
|
• in calf muscles after tamoxifen administration and 28 days after hind-limb ischemia (HLI) induction
• same muscle damage as wildtype in calf muscles after tamoxifen administration and 1 day after hind-limb ischemia (HLI) induction
|
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• basally localized glomeruloid malformations in hemorrhagic areas of the CNS
|
|
• central nervous system (CNS) angiogenic arrest
|
|
• stated to fully recapitulate the phenotype seen in mice homozygous for Gpr124tm1Cjku
• forebrain hemorrhage
|
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• delayed superficial blood vessel growth and defects in vertical blood vessel growth in retina at age P9 after tamoxifen administration from age P1-P4
• absent secondary and tertiary blood vessels in deeper layers of retina at age P9 after tamoxifen administration from age P1-P4
• mild delay of blood vessel growth in deeper layers of retina at age P13 after tamoxifen administration from age P6
|
|
• delayed superficial blood vessel growth and defects in vertical blood vessel growth at age P9 after tamoxifen administration from age P1-P4
• absent secondary and tertiary blood vessels in deeper layers at age P9 after tamoxifen administration from age P1-P4
• mild delay of blood vessel growth in deeper layers at age P13 after tamoxifen administration from age P6
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| exudative vitreoretinopathy | DOID:0050535 |
OMIM:PS133780 |
J:328283 | |
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• embryos exposed to tamoxifen between E7.5 and E9.5
|
|
• retarded growth in 25% of embryos at E11.5
|
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• decreased embryonic vessels in the labyrinth at E10.5
|
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• yolk sac vascularization is altered
|
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• decreased embryonic vessels in the labyrinth at E10.5
|
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• yolk sac vascularization is altered
|
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• retarded growth in 25% of embryos at E11.5
|
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• increased early mortality
|
|
• cerebral cavernous malformations develop by one month after tamoxifen treatment at 1 day of age
• additional abnormalities include capillary telangiectasias, isolated caverns and multiple back to back caverns, thrombosis, hemorrhage, formation of secondary channels
• attenuation of endothelial cells in larger secondary channels but no gaps
|
|
• foot processes are missing
|
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• cerebral cavernous malformations develop by one month after tamoxifen treatment at 1 day of age
• additional abnormalities include capillary telangiectasias, isolated caverns and multiple back to back caverns, thrombosis, hemorrhage, formation of secondary channels
• attenuation of endothelial cells in larger secondary channels but no gaps
|
|
• foci of mononuclear inflammatory cells also seen
|
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• foot processes are missing
|
|
• foci of mononuclear inflammatory cells also seen
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| cerebral cavernous malformation 3 | DOID:0060671 |
OMIM:603285 |
J:173947 | |
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• increased early mortality
|
|
• cerebral cavernous malformations develop as early as two months after tamoxifen treatment at 1 day of age
• most mice have lesions at 4 months and all mice at 6 months
• additional abnormalities include capillary telangiectasias, isolated caverns and multiple back to back caverns, thrombosis, hemorrhage, formation of secondary channels
• attenuation of endothelial cells in larger secondary channels but no gaps
|
|
• foot processes are missing
|
|
• cerebral cavernous malformations develop as early as two months after tamoxifen treatment at 1 day of age
• most mice have lesions at 4 months and all mice at 6 months
• additional abnormalities include capillary telangiectasias, isolated caverns and multiple back to back caverns, thrombosis, hemorrhage, formation of secondary channels
• attenuation of endothelial cells in larger secondary channels but no gaps
|
|
• foci of mononuclear inflammatory cells also seen
|
|
• foot processes are missing
|
|
• foci of mononuclear inflammatory cells also seen
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| cerebral cavernous malformation 2 | DOID:0060670 |
OMIM:603284 |
J:173947 | |
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• postnatal tamoxifen treatment from P1-P3 results in decreased angiogenesis in the retina assessed at P6; significantly decreased vascular branching and endothelial cell coverage at P6
|
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• delayed superficial blood vessel growth in retina at age P7 after tamoxifen administration from age P1-P4
|
|
• local blood vessel hyperdensity in retina at age P7 after tamoxifen administration from age P1-P4
|
|
• extensive blood leakage in retina at age P7 after tamoxifen administration from age P1-P4
|
|
• delayed superficial blood vessel growth at age P7 after tamoxifen administration from age P1-P4
• local blood vessel hyperdensity at age P7 after tamoxifen administration from age P1-P4
• extensive blood leakage at age P7 after tamoxifen administration from age P1-P4
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| exudative vitreoretinopathy | DOID:0050535 |
OMIM:PS133780 |
J:328283 | |
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• reduced postnatal vessel outgrowth with reduced branching in tamoxifen-treated mice
|
|
• reduced in the retina of tamoxifen-treated mice with reduced number of tip cells
• however, vessel pruning is normal
|
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• in tamoxifen-treated mice
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• reduced postnatal vessel outgrowth with reduced branching in tamoxifen-treated mice
|
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• in tamoxifen-treated mice
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• in tamoxifen-treated mice
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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 04/16/2024 MGI 6.23 |
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