Phenotypes associated with this allele
Allelic
Composition |
Pdgfrbtm1Sor/Pdgfrbtm1Sor
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Genetic
Background |
either: 129S7/SvEvBrd-Pdgfrbtm1Sor or (involves: 129S7/SvEvBrd * C57BL/6J) |
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdgfrbtm1Sor mutation
(1 available);
any
Pdgfrb mutation
(84 available)
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mortality/aging
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• some embryos are dead around E16-18.5
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• majority of mutants die perinatally
• cesarean delivered pups die within a matter of minutes of delivery
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homeostasis/metabolism
hematopoietic system
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• significantly elevated number of nucleated erythrocytes
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cardiovascular system
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• capillaries within the renal glomerulus are very scarce
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• some cesarean delivered pups suddenly lose most of their blood through the cut in the umbilical cord
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• kidney shows specks of blood
• blood cells fill up the capsule space
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• the capillary tuft normally consisting of podoctyes and mesangial cells is missing in every glomerulus and is replaced with blood cells filling up the capsule space
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renal/urinary system
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• capillaries within the renal glomerulus are very scarce
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• kidney shows specks of blood
• blood cells fill up the capsule space
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• the capillary tuft normally consisting of podoctyes and mesangial cells is missing in every glomerulus and is replaced with blood cells filling up the capsule space
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• absence, or significant paucity, of mesangial cells
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integument
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• cesarean delivered pups are pale
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• at E16 and E18.5, about 1/3 of embryos exhibit purpura, an accumulation of blood under the surface of the dermis
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cellular
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• absence, or significant paucity, of mesangial cells
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdgfrbtm1Sor mutation
(1 available);
any
Pdgfrb mutation
(84 available)
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cardiovascular system
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• perivascular cells are missing from angiogenic sprouts
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• ES cells fail to respond to application of Pdgfb by forming a peripheral capillary plexus as wild-type cells do
• however, formation of Pdgf-independent vascular structures is normal
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdgfrbtm1Sor mutation
(1 available);
any
Pdgfrb mutation
(84 available)
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cardiovascular system
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• mean blood vessel diameter is increased by 25%
• exhibit regional dilations along the blood vessel length that are not seen in wild-type
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• the circumferential length of endothelial cell junctions is reduced by 25%, indicating that endothelial cells, on average, are more densely packed in capillary walls
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• exhibit endothelial cell hyperplasia in the brain from E11.5 on, however hyperplasia is not seen in the perineural vascular plexus surrounding the brain
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• 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
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• fetal vessels are dilated to a varying degree in placentas; seen by E13.5
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• the fetal vessel parametric length and area is increased relative to the maternal lacunas in the labyrinthine layer at E17
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embryo
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• fetal vessels are dilated to a varying degree in placentas; seen by E13.5
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• the fetal vessel parametric length and area is increased relative to the maternal lacunas in the labyrinthine layer at E17
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• nonendothelial labyrinthine cell number, dominated by the trophoblasts, is reduced by about 40%
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Allelic
Composition |
Pdgfrbtm1Sor/Pdgfrbtm5Sor
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Genetic
Background |
either: (involves: 129S4/SvJaeSor * 129S7/SvEvBrd) or (involves: 129S4/SvJaeSor * 129S7/SvEvBrd * C57BL/6) |
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdgfrbtm1Sor mutation
(1 available);
any
Pdgfrb mutation
(84 available)
Pdgfrbtm5Sor mutation
(0 available);
any
Pdgfrb mutation
(84 available)
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mortality/aging
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• 58% die before 21 days of age
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growth/size/body
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• the heart-to-body weight ratio exceeds controls by 105%
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• mutants that survive to adulthood display kidneys that often contain 1-5 simple renal cysts
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• mutants that die before 21 days of age are typically half the size of control littermates
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cardiovascular system
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• mutants crossed to a beta-galactosidase transgene in pericytes show that retinas contain highly disorganized vasculature, prominent areas of hemorrhaging and a paucity of pericytes both at the center and out toward the periphery of the tissue
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• 9 of 11 exhibit kidney microaneurysms
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• exhibit loss of pericytes at the retinal microvasculature
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• myofibers are highly disorganized
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• the heart-to-body weight ratio exceeds controls by 105%
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• show thickening of the left ventricle
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• show distension of the right ventricle
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• most likely die due to hemorrhaging as autopsy often shows clots of blood surrounding the heart
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• retinas contain prominent areas of hemorrhaging
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renal/urinary system
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• 9 of 11 exhibit kidney microaneurysms
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• mutants that survive to adulthood display kidneys that often contain 1-5 simple renal cysts
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• 9 of 11 exhibit many abnormal glomeruli (60-80%) that lack the formation of a capillary tuft at E18.5
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• mesangial cell development is inhibited
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• 30-60% of glomeruli are damaged by glomerulosclerosis; observe as early as 28 days after birth and fully penetrant by 3 months of age
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vision/eye
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• as early as 27 days following birth, observe a cotton-like opacity in the center of the eye and develop a proliferative retinopathy
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• mutants crossed to a beta-galactosidase transgene in pericytes show that retinas contain highly disorganized vasculature, prominent areas of hemorrhaging and a paucity of pericytes both at the center and out toward the periphery of the tissue
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• retinas contain prominent areas of hemorrhaging
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• retina is detached from the posterior of the eye, migrates into the vitreous body, and loses its normal layered appearance, a phenotype that resembles diabetic retinopathy
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muscle
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• myofibers are highly disorganized
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cellular
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• mesangial cell development is inhibited
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Allelic
Composition |
Pdgfrbtm1Sor/Pdgfrbtm6Sor
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Genetic
Background |
either: (involves: 129S4/SvJaeSor * 129S7/SvEvBrd) or (involves: 129S4/SvJaeSor * 129S7/SvEvBrd * C57BL/6) |
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdgfrbtm1Sor mutation
(1 available);
any
Pdgfrb mutation
(84 available)
Pdgfrbtm6Sor mutation
(0 available);
any
Pdgfrb mutation
(84 available)
|
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normal phenotype
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• viable and develop normally
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdgfrbtm1Sor mutation
(1 available);
any
Pdgfrb mutation
(84 available)
Pdgfrbtm8Sor mutation
(0 available);
any
Pdgfrb mutation
(84 available)
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cardiovascular system
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• 100% of mutants contain fewer discontinuous blood vessels and overgrowth of retinal cells, however severity of this phenotype is variable
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• 100% of mutants contain fewer discontinuous blood vessels and overgrowth of retinal cells, however severity of this phenotype is variable
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• 70-92% decrease in pericytes in the kidney, heart, and eye
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• sometimes exhibit hemorrhage in the eye
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vision/eye
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• sometimes exhibit hemorrhage in the eye
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• 100% of mutants contain fewer discontinuous blood vessels and overgrowth of retinal cells, however severity of this phenotype is variable
|
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|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdgfrbtm1Sor mutation
(1 available);
any
Pdgfrb mutation
(84 available)
Pdgfrbtm9Sor mutation
(0 available);
any
Pdgfrb mutation
(84 available)
|
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cardiovascular system
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• 100% of mutants contain fewer discontinuous blood vessels and overgrowth of retinal cells, however severity of this phenotype is variable
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• 100% of mutants contain fewer discontinuous blood vessels and overgrowth of retinal cells, however severity of this phenotype is variable
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• 70-92% decrease in pericytes in the heart, kidney, and eye
• reduction in vascular smooth muscle cells/pericytes in the thoracic region of E14.5 mutants
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• sometimes exhibit hemorrhage in the eye
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vision/eye
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• sometimes exhibit hemorrhage in the eye
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• 100% of mutants contain fewer discontinuous blood vessels and overgrowth of retinal cells, however severity of this phenotype is variable
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cardiovascular system
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• 70-92% decrease in pericytes in the kidney and heart
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| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pdgfrbtm1Sor mutation
(1 available);
any
Pdgfrb mutation
(84 available)
Pdgfrbtm4Sor mutation
(0 available);
any
Pdgfrb mutation
(84 available)
|
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cardiovascular system
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• 70-92% decrease in pericytes in the kidney and heart
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hematopoietic system
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N |
• anemia seen in Myo1eGt(ROSA)74Sor is rescued
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renal/urinary system
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• glomeruli are often swollen and degraded
• smooth muscle cell number is reduced in the glomeruli
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• decrease in kidney function
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muscle
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• smooth muscle cell number is reduced in the glomeruli
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homeostasis/metabolism
skeleton
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• skeletal defects are more severe than in Schip1Gt(ROSA)77Sor homozygotes
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renal/urinary system
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• glomeruli are often swollen and degraded
• smooth muscle cell number is reduced in the glomeruli
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• decrease in kidney function
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homeostasis/metabolism
muscle
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• smooth muscle cell number is reduced in the glomeruli
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vision/eye
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• the average number of vitreous cells in E14.5-E15.5 embryos is decreased compared to mice homozygous for Cdkn2atm1Cjs and heterozygous for Pdgfrbtm1Sor
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Analysis Tools
