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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Pdgfbtm1Cbet
targeted mutation 1, Christer Betsholtz
MGI:1933334
Summary 7 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Pdgfbtm1Cbet/Pdgfbtm1Cbet involves: 129P2/OlaHsd * C57BL/6 MGI:2450095
ht2
Pdgfbtm1Cbet/Pdgfbtm3.1Cbet involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ MGI:3694682
cn3
Pdgfbtm1Cbet/Pdgfbtm2Cbet
Tg(Tie1-cre)9Ref/0
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA MGI:2449991
cn4
Gt(ROSA)26Sortm1(PDGFB)Cbet/Gt(ROSA)26Sor+
Pdgfbtm1Cbet/Pdgfbtm1Cbet
Tg(Fabp4-lacZ)4Mosh/0
Tg(Tek-cre)1Ywa/0
involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * FVB/N * SJL MGI:4849465
cn5
Gt(ROSA)26Sortm1(PDGFB)Cbet/Gt(ROSA)26Sortm1(PDGFB)Cbet
Pdgfbtm1Cbet/Pdgfbtm1Cbet
Tg(Fabp4-lacZ)4Mosh/0
Tg(Tek-cre)1Ywa/0
involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * FVB/N * SJL MGI:4849467
cn6
Gt(ROSA)26Sortm1(PDGFB)Cbet/Gt(ROSA)26Sor+
Pdgfbtm1Cbet/Pdgfbtm1Cbet
Tg(Tek-cre)1Ywa/0
involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * SJL MGI:4849464
cn7
Gt(ROSA)26Sortm1(PDGFB)Cbet/Gt(ROSA)26Sortm1(PDGFB)Cbet
Pdgfbtm1Cbet/Pdgfbtm1Cbet
Tg(Tek-cre)1Ywa/0
involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * SJL MGI:4849466


Genotype
MGI:2450095
hm1
Allelic
Composition
Pdgfbtm1Cbet/Pdgfbtm1Cbet
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdgfbtm1Cbet mutation (0 available); any Pdgfb mutation (15 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Abnormal endothelial cell morphology and increased caveolae formation in Pdgfbtm1Cbet/Pdgfbtm1Cbet brains

mortality/aging
• ~25% of homozygotes die 1-2 days prior to birth (E17-E18.5) with a hemorrhagic and edematous phenotype
• ~75% of homozygotes die at birth

renal/urinary system
• at E17.5 or later, homozygotes exhibit a spotty kidney phenotype
• the mutant Bowman's capsule space contains blood-filled aneurysm-like structures
• at E17.5 or later, homozygotes exhibit abnormal mature glomerular bodies ("red spots") in the inner part of the cortex, with capillary aneurysms replacing the glomerular tufts
• at E17.5 or later, mutant glomeruli show absence or reduction of mesangial cells in the presence of a morphologically normal basement membrane
• at E17.5 or later, mutant glomeruli may show absence of mesangial cells
• at E17.5 or later, homozygotes exhibit smaller kidneys
• at E17.5 or later, homozygotes display empty urinary bladders, suggesting kidney dysfunction

cardiovascular system
• at E17.5 or later, homozygotes exhibit dilated thoracic large arteries as well as dilated mesenteric blood vessels (J:20017)
• however, no hypoplasia of smooth muscle cells is observed (J:20017)
• exhibit regional dilations along the blood vessel length that are not seen in wild-type (J:78912)
• exhibit a 2.5-fold increase in the number of caveolae in brain vessels (J:78912)
• at E17.5 or later, homozygotes display dilated thoracic large arteries
• at E16.5-E19, homozygotes display a dilated aorta with an extended, thinner aortic muscular wall
• at E16.5-E19, homozygotes display a dilated aorta with an extended, thinner aortic muscular wall
• endothelial cells in the brain exhibit a luminal surface that has multiple processes projecting into the lumen of the vessel
• thickness of endothelium varies considerably; in some areas the cytoplasm of the cells is very thin and in others it is thicker than in wild-type
• exhibit endothelial cell hyperplasia in the brain from E11.5 on, however hyperplasia is not seen in the perineural vascular plexus surrounding the brain
• exhibit increased capillary diameter in the brain
• the mutant Bowman's capsule space contains blood-filled aneurysm-like structures
• exhibit loss and altered organization of pericytes in placentas; pericytes are found in the labyrinthine layer but are reduced by about 50% and are completely absent from the spongiotrophoblast layer
• pericytes are displaced from multicellular core aggregates to scattered sites in the walls of dilated fetal vessels
• fetal vessels are dilated to a varying degree in placentas; seen by E13.5
• the fetal vessel parametric length and area is increased relative to the maternal lacunas in the labyrinthine layer at E17
• homozygotes dying at E17-E18.5 display dilated and congested subpericardial blood vessels
• at E17.5 or later, homozygotes exhibit enlarged and malformed hearts, with extreme dilation of all chambers and of the thoracic aortae
• homozygotes dying at E17-E18.5 show hypertrabeculated and thin-walled regions in the right ventricle
• at E19, most live embryos exhibit hemorrhages in subcutaneous regions, periscapular brown fat and the liver

hematopoietic system
• at E19, homozygotes display reduced hematocrits relative to wild-type mice
• at E19, homozygotes display reduced hemoglobin values relative to wild-type mice
• at E19, homozygotes exhibit a ~20% increase in MCV
• at E19, homozygotes show a ~85% reduction in thrombocyte counts relative to wild-type mice
• no differences in granulocyte, lymphocyte, and monocyte differential cell counts are observed

respiratory system
• all live homozygotes delivered by Cesarian section gasp for air a few times, then cease to breathe and die

liver/biliary system
• at E17.5 or later, homozygotes exhibit reduced liver volume

homeostasis/metabolism
• homozygotes dying at E17-E18.5 display a generalized subcutaneous edema

embryo
• fetal vessels are dilated to a varying degree in placentas; seen by E13.5
• the fetal vessel parametric length and area is increased relative to the maternal lacunas in the labyrinthine layer at E17
• nonendothelial labyrinthine cell number, dominated by the trophoblasts, is reduced by about 40%

nervous system
• many glia cells that are juxtaposed to the endothelial cells of vessels exhibit prominent swelling

muscle
• at E16.5-E19, homozygotes display a dilated aorta with an extended, thinner aortic muscular wall

integument
• homozygotes dying at E17-E18.5 display a generalized subcutaneous edema

growth/size/body
• at E17.5 or later, homozygotes exhibit enlarged and malformed hearts, with extreme dilation of all chambers and of the thoracic aortae

cellular
• at E17.5 or later, mutant glomeruli show absence or reduction of mesangial cells in the presence of a morphologically normal basement membrane
• at E17.5 or later, mutant glomeruli may show absence of mesangial cells




Genotype
MGI:3694682
ht2
Allelic
Composition
Pdgfbtm1Cbet/Pdgfbtm3.1Cbet
Genetic
Background
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdgfbtm1Cbet mutation (0 available); any Pdgfb mutation (15 available)
Pdgfbtm3.1Cbet mutation (0 available); any Pdgfb mutation (15 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging

cardiovascular system
• at E15.5, the number of pericytes in the forebrain is reduced to about 25% of normal




Genotype
MGI:2449991
cn3
Allelic
Composition
Pdgfbtm1Cbet/Pdgfbtm2Cbet
Tg(Tie1-cre)9Ref/0
Genetic
Background
involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdgfbtm1Cbet mutation (0 available); any Pdgfb mutation (15 available)
Pdgfbtm2Cbet mutation (1 available); any Pdgfb mutation (15 available)
Tg(Tie1-cre)9Ref mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
muscle
• thin myocardium is seen at E18.5, however this abnormality is normalized by 1 month of age

cardiovascular system
• E15.5 embryos show irregular capillary diameter with microaneurysms in the brain
• capillaries in the striatum with associated pericytes are straighter and more uniform in diameter
• in the cerebellum, see a reduction of capillary density in the gray matter and the presence of numerous enlarged capillaries
• in the striatum, capillary density is lower, but numerous tortuous capillaries with increased diameter are seen in capillary regions not associated with pericytes
• in the cerebellum, see a reduction of capillary density in the gray matter and the presence of numerous enlarged capillaries
• E18.5 glomeruli show dilation of the remaining capillary loops
• 3-week old mutants show an increase in the diameter of individual capillary loops
• in the striatum, capillary density is lower, but numerous tortuous capillaries with increased diameter are seen in capillary regions not associated with pericytes
• microaneurysm formation in the brain
• in mutants with greater than 50% of normal overall CNS pericyte density, the retinal vasculature displays irregular microvessel diameter, microaneurysms, and increased vascular regression
• in mutants with less than 50% of normal pericyte density, the retinas develop regions with massive increase of abnormal vessels extending into the vitreous and choroid
• exhibit a variable reduction in pericyte density in CNS vessels, affecting arteries, veins and capillaries (J:78544)
• retinas in mutants with the lowest overall CNS pericyte density however display focal regions of increased pericyte density (J:78544)
• exhibit a significant reduction in pericyte density in E15.5 embryos that persists into adulthood (J:89186)
• placental defects at E18.5 include dilation of both fetal and maternal vessels due to a reduction in the number of pericytes and trophoblasts
• thin myocardium is seen at E18.5, however this abnormality is normalized by 1 month of age
• show scattered small hemorrhages deep in the cerebral parenchyma; bleeding seems to start from capillary branching points

vision/eye
• mutants with less than 52% of the normal pericyte density in the cerebellum show typical hallmarks of proliferative retinopathy, affecting at least one eye
• retinas of mutants with the lowest overall CNS pericyte density are contracted and often attached to the retinal pigment epithelial cells and the lens
• in mutants with greater than 50% of normal overall CNS pericyte density, the retinal vasculature displays irregular microvessel diameter, microaneurysms, and increased vascular regression
• in mutants with less than 50% of normal pericyte density, the retinas develop regions with massive increase of abnormal vessels extending into the vitreous and choroid
• regions with high pericyte density show loss of organization of the neural layers and folding of the photoreceptor layer producing typical photoreceptor rosette profiles

embryo
• placental defects at E18.5 include dilation of both fetal and maternal vessels due to a reduction in the number of pericytes and trophoblasts

homeostasis/metabolism
• mutants older than 12 months develop mild but significant increases in albumin content in urine

nervous system
• microaneurysm formation in the brain
• show scattered small hemorrhages deep in the cerebral parenchyma; bleeding seems to start from capillary branching points
• postnatal brains show increased density of microglial cells at sites of bleeding
• postnatal brains show increased density of microglial cells and upregulated expression of glial fibrillary acidic protein at sites of bleeding, hallmarks of reactive gliosis

renal/urinary system
• mutants older than 12 months develop mild but significant increases in albumin content in urine
• E18.5 glomeruli show a reduction in tuft complexity and dilation of the remaining capillary loops
• 3-week old mutants show glomerular dilation, both an increased glomerulus diameter and an increase in the diameter of the individual capillary loops, however by 6 and 21 months of age, no signs of increased glomerular pathology are seen
• E18.5 glomeruli show dilation of the remaining capillary loops
• 3-week old mutants show an increase in the diameter of individual capillary loops
• most glomeruli show a reduced mesangial core, although some completely lack mesangial cells at E18.5
• the mesangial deficiency is largely corrected at 3 weeks of age

hematopoietic system
• postnatal brains show increased density of microglial cells at sites of bleeding

immune system
• postnatal brains show increased density of microglial cells at sites of bleeding

cellular
• most glomeruli show a reduced mesangial core, although some completely lack mesangial cells at E18.5
• the mesangial deficiency is largely corrected at 3 weeks of age




Genotype
MGI:4849465
cn4
Allelic
Composition
Gt(ROSA)26Sortm1(PDGFB)Cbet/Gt(ROSA)26Sor+
Pdgfbtm1Cbet/Pdgfbtm1Cbet
Tg(Fabp4-lacZ)4Mosh/0
Tg(Tek-cre)1Ywa/0
Genetic
Background
involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * FVB/N * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm1(PDGFB)Cbet mutation (0 available); any Gt(ROSA)26Sor mutation (935 available)
Pdgfbtm1Cbet mutation (0 available); any Pdgfb mutation (15 available)
Tg(Fabp4-lacZ)4Mosh mutation (1 available)
Tg(Tek-cre)1Ywa mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• mural cell (pericytes and vascular smooth muscle cells) densities are lower than in wild-type mice




Genotype
MGI:4849467
cn5
Allelic
Composition
Gt(ROSA)26Sortm1(PDGFB)Cbet/Gt(ROSA)26Sortm1(PDGFB)Cbet
Pdgfbtm1Cbet/Pdgfbtm1Cbet
Tg(Fabp4-lacZ)4Mosh/0
Tg(Tek-cre)1Ywa/0
Genetic
Background
involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * FVB/N * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm1(PDGFB)Cbet mutation (0 available); any Gt(ROSA)26Sor mutation (935 available)
Pdgfbtm1Cbet mutation (0 available); any Pdgfb mutation (15 available)
Tg(Fabp4-lacZ)4Mosh mutation (1 available)
Tg(Tek-cre)1Ywa mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
N
• mural cell (pericytes and vascular smooth muscle cells) densities are close to normal




Genotype
MGI:4849464
cn6
Allelic
Composition
Gt(ROSA)26Sortm1(PDGFB)Cbet/Gt(ROSA)26Sor+
Pdgfbtm1Cbet/Pdgfbtm1Cbet
Tg(Tek-cre)1Ywa/0
Genetic
Background
involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm1(PDGFB)Cbet mutation (0 available); any Gt(ROSA)26Sor mutation (935 available)
Pdgfbtm1Cbet mutation (0 available); any Pdgfb mutation (15 available)
Tg(Tek-cre)1Ywa mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• mice exhibit increased blood-brain barrier (BBB) permeability compared with wild-type mice
• however, imatinib-treatment reverses increased BBB permeability without improving blood vessel morphology
• astrocyte end-feet are mis-localized compared to in wild-type mice

cardiovascular system
• capillary diameter is increased compared to in wild-type mice
• capillary density is reduced compared to in wild-type mice
• pericyte coverage is less than in wild-type mice but not as severely as in Pdgfbtm3.1Cbet homozygotes
• mice exhibit increased blood-brain barrier (BBB) permeability compared with wild-type mice
• however, imatinib-treatment reverses increased BBB permeability without improving blood vessel morphology




Genotype
MGI:4849466
cn7
Allelic
Composition
Gt(ROSA)26Sortm1(PDGFB)Cbet/Gt(ROSA)26Sortm1(PDGFB)Cbet
Pdgfbtm1Cbet/Pdgfbtm1Cbet
Tg(Tek-cre)1Ywa/0
Genetic
Background
involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm1(PDGFB)Cbet mutation (0 available); any Gt(ROSA)26Sor mutation (935 available)
Pdgfbtm1Cbet mutation (0 available); any Pdgfb mutation (15 available)
Tg(Tek-cre)1Ywa mutation (6 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• mice exhibit increased blood-brain barrier (BBB) permeability compared with wild-type mice
• however, imatinib-treatment reverses increased BBB permeability
• astrocyte end-feet are mis-localized compared to in wild-type mice

cardiovascular system
• capillary diameter is increased compared to in wild-type mice
• capillary density is reduced compared to in wild-type mice
• pericyte coverage is less than in wild-type mice but not as severely as in Pdgfbtm3.1Cbet homozygotes
• mice exhibit increased blood-brain barrier (BBB) permeability compared with wild-type mice
• however, imatinib-treatment reverses increased BBB permeability





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last database update
03/19/2024
MGI 6.23
The Jackson Laboratory