Strain Name:

129S-Pdgfrbtm1Sor/J

Stock Number:

007846

Availability:

Repository- Live
Homozygous embryos exhibit purpura, edema, anemia, polychromasia, anisocytosis and abnormal kidney development. Some homozygous embryos do not survive past E18.5. This mutant mouse strain may be useful in studies of hematopoiesis, kidney development and and cellular signaling during development.

Description

Strain Information

Type Mutant Stock; Mutant Strain; Targeted Mutation;
Mating SystemHeterozygote x +/+ sibling         (Female x Male)
Specieslaboratory mouse
GenerationN?+F1 (08-JAN-08)
 
Donating Investigator Philippe Soriano,   Fred Hutchinson Cancer Research Center

Description
One third of homozygous embryos, aged E16 to E18.5, exhibit purpura and edema with some embryos in this group dying at this stage. Homozygous embryos delivered by Caesarean section at E18.5 die within minutes. Homozygotes exhibit anemia, elevated numbers of nucleated erythrocytes, polychromasia, irregularly shaped mature erythrocytes (anisocytosis), and hemorrhaging. Glomerular capillary tufts are absent and the capsule space is filled with blood cells. No gene product (protein) is detected by Western blot analysis of total protein. A truncated transcript presumed to be due to exon skipping is detected by Northern blot analysis. This mutant mouse strain may be useful in studies of hematopoiesis, kidney development and and cellular signaling during development.

Development
A targeting vector containing a PGKneo cassette was used to disrupt 1.8 kb of sequence encoding the signal peptide and the second immunoglobulin domain of the targeted gene.

The construct was electroporated into 129S7/SvEvBrd-Hprt1+ derived AB1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into recipient blastocysts. Heterozygous mice were maintained on a 129S4/SvJaeSor background prior to arrival at The Jackson Laboratory. The mice were then crossed to 129S1/SvImJ (Stock No. 002448) for one generation.

Control Information

  Control
   Wild-type from the colony
   002448 129S1/SvImJ
 
  Considerations for Choosing Controls

Phenotype

Phenotype Information

Mammalian Phenotype Terms assigned by genotype

Pdgfrbtm1Sor/Pdgfrbtm1Sor

        either: 129S7/SvEvBrd-Pdgfrbtm1Sor or (involves: 129S7/SvEvBrd * C57BL/6J)
  • lethality-prenatal/perinatal
  • lethality throughout fetal growth and development (MGI Ref ID J:20016)
    • some embryos are dead around E16-18.5
  • perinatal lethality (MGI Ref ID J:20016)
    • majority of mutants die perinatally
    • cesarean delivered pups die within a matter of minutes of delivery
  • homeostasis/metabolism phenotype
  • hydrops fetalis (MGI Ref ID J:20016)
    • the 1/3 of embryos that exhibit purpura are also edematous
  • skin/coat/nails phenotype
  • pallor (MGI Ref ID J:20016)
    • cesarean delivered pups are pale
  • hematopoietic system phenotype
  • abnormal erythrocyte morphology (MGI Ref ID J:20016)
    • anisocytosis (MGI Ref ID J:20016)
    • increased erythrocyte cell number (MGI Ref ID J:20016)
      • significantly elevated number of nucleated erythrocytes
    • poikilocytosis (MGI Ref ID J:20016)
      • schistocytosis (MGI Ref ID J:20016)
    • polychromatophilia (MGI Ref ID J:20016)
  • anemia (MGI Ref ID J:20016)
  • decreased platelet cell number (MGI Ref ID J:20016)
  • cardiovascular system phenotype
  • abnormal blood circulation (MGI Ref ID J:20016)
    • about 1/3 of embryos exhibit purpura, an accumulation of blood under the surface of the dermis, at E16 and E18.5
    • hemorrhage (MGI Ref ID J:20016)
      • some cesarean delivered pups suddenly lose most of their blood through the cut in the umbilical cord
      • kidney hemorrhage (MGI Ref ID J:20016)
        • kidney shows specks of blood
  • abnormal kidney vasculature (MGI Ref ID J:20016)
    • capillaries within the renal glomerulus are very scarce
  • abnormal venule morphology (MGI Ref ID J:20016)
    • dilation of veinules
  • renal/urinary system phenotype
  • abnormal kidney vasculature (MGI Ref ID J:20016)
    • capillaries within the renal glomerulus are very scarce
  • abnormal renal glomerulus morphology (MGI Ref ID J:20016)
    • 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
    • capillaries within the glomerulus are very scarce
    • abnormal mesangial cell (MGI Ref ID J:20016)
      • absent
    • absent podocytes (MGI Ref ID J:20016)
      • absent
  • kidney hemorrhage (MGI Ref ID J:20016)
    • kidney shows specks of blood

Pdgfrbtm1Sor/Pdgfrbtm1Sor

        involves: 129S7/SvEvBrd
  • cardiovascular system phenotype
  • abnormal angiogenesis (MGI Ref ID J:138069)
    • perivascular cells are missing from angiogenic sprouts
    • decreased angiogenesis (MGI Ref ID J:138069)
      • 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

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Pdgfrbtm1Sor/Pdgfrbtm1Sor

        involves: 129S7/SvEvBrd * C57BL/6
  • cardiovascular system phenotype
  • abnormal vasculature (MGI Ref ID J:78912)
    • mean blood vessel diameter is increased by 25%
    • exhibit regional dilations along the blood vessel length that are not seen in wild-type
    • abnormal pericyte morphology (MGI Ref ID J:56682)
      • 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
    • abnormal vascular endothelial cell morphology (MGI Ref ID J:78912)
      • the circumferential length of endothelial cell junctions is reduced by 25%, indicating that endothelial cells, on average, are more densely packed in capillary walls
      • increased vascular endothelial cell number (MGI Ref ID J:78912)
        • exhibit endothelial cell hyperplasia in the brain from E11.5 on, however hyperplasia is not seen in the perineural vascular plexus surrounding the brain
  • embryogenesis phenotype
  • abnormal placenta vasculature (MGI Ref ID J:56682)
    • fetal vessels are dilated to a varying degree in placentas; seen by E13.5
    • abnormal placental labyrinth vasculature morphology (MGI Ref ID J:56682)
      • the fetal vessel parametric length and area is increased relative to the maternal lacunas in the labyrinthine layer at E17
  • abnormal trophoblast layer morphology (MGI Ref ID J:56682)
    • nonendothelial labyrinthine cell number, dominated by the trophoblasts, is reduced by about 40%

Research Applications

This mouse can be used to support research in many areas including:

Developmental Biology Research
Internal/Organ Defects (kidney)
Perinatal Lethality (Homozygous)

Hematological Research
Hematopoietic Defects

Genes & Alleles

Gene & Allele Information

Allele Symbol Pdgfrbtm1Sor
Allele Name targeted mutation 1, Philippe Soriano
Common Name(s) PDGFR-beta-;
Strain of Origin129S7/SvEvBrd-Hprt1<+>
ES Cell Line NameAB1
ES Cell Line Strain129S7/SvEvBrd-Hprt1<+>
Gene Symbol and Name Pdgfrb, platelet derived growth factor receptor, beta polypeptide
Chromosome 18
Gene Common Name(s) AI528809; CD140B; JTK12; PDGF beta chain; PDGF-R-beta; PDGFR; PDGFR1; Pdgfr; expressed sequence AI528809; platelet derived growth factor receptor;
Molecular Note A neomycin resistance cassette replaced 1.8 kb of genomic sequence, which encodes part of the signal peptide and the second immunoglobulin domain. Northern blot analysis detected a shorter transcript in homozygous mutant mice that was presumed to be dueto exon skipping. Western blot analysis of total protein failed to detect any encoded protein in homozygous mice. [MGI Ref ID J:20016]

Genotyping

Genotyping Information

Genotyping Protocols

Pdgfrbtm1Sor, SEP PCR, vers. 1

Helpful Links

Optimizing PCR Protocols

References

References

Selected Reference(s)

Soriano P. 1994. Abnormal kidney development and hematological disorders in PDGF beta-receptor mutant mice. Genes Dev 8(16):1888-96. [PubMed: 7958864]  [MGI Ref ID J:20016]

Additional References

Pdgfrbtm1Sor related

Basciani S; Mariani S; Arizzi M; Brama M; Ricci A; Betsholtz C; Bondjers C; Ricci G; Catizone A; Galdieri M; Spera G; Gnessi L. 2004. Expression of platelet-derived growth factor (PDGF) in the epididymis and analysis of the epididymal development in PDGF-A, PDGF-B, and PDGF receptor beta deficient mice. Biol Reprod 70(1):168-77. [PubMed: 14522834]  [MGI Ref ID J:89838]

Bondjers C; He L; Takemoto M; Norlin J; Asker N; Hellstrom M; Lindahl P; Betsholtz C. 2006. Microarray analysis of blood microvessels from PDGF-B and PDGF-Rbeta mutant mice identifies novel markers for brain pericytes. FASEB J 20(10):1703-5. [PubMed: 16807374]  [MGI Ref ID J:111339]

Hellstrom M; Gerhardt H; Kalen M; Li X; Eriksson U; Wolburg H; Betsholtz C. 2001. Lack of pericytes leads to endothelial hyperplasia and abnormal vascular morphogenesis. J Cell Biol 153(3):543-53. [PubMed: 11331305]  [MGI Ref ID J:78912]

Hellstrom M; Kal n M; Lindahl P; Abramsson A; Betsholtz C. 1999. Role of PDGF-B and PDGFR-beta in recruitment of vascular smooth muscle cells and pericytes during embryonic blood vessel formation in the mouse. Development 126(14):3047-55. [PubMed: 10375497]  [MGI Ref ID J:20328]

Klinghoffer RA; Mueting-Nelsen PF; Faerman A; Shani M; Soriano P. 2001. The two PDGF receptors maintain conserved signaling in vivo despite divergent embryological functions. Mol Cell 7(2):343-54. [PubMed: 11239463]  [MGI Ref ID J:67735]

Lindahl P; Hellstrom M; Kalen M; Karlsson L; Pekny M; Pekna M; Soriano P; Betsholtz C. 1998. Paracrine PDGF-B/PDGF-Rbeta signaling controls mesangial cell development in kidney glomeruli. Development 125(17):3313-22. [PubMed: 9693135]  [MGI Ref ID J:50064]

Ohlsson R; Falck P; Hellstrom M; Lindahl P; Bostrom H; Franklin G; Ahrlund-Richter L; Pollard J; Soriano P; Betsholtz C. 1999. PDGFB regulates the development of the labyrinthine layer of the mouse fetal placenta. Dev Biol 212(1):124-36. [PubMed: 10419690]  [MGI Ref ID J:56682]

Rolny C; Nilsson I; Magnusson P; Armulik A; Jakobsson L; Wentzel P; Lindblom P; Norlin J; Betsholtz C; Heuchel R; Welsh M; Claesson-Welsh L. 2006. Platelet-derived growth factor receptor-beta promotes early endothelial cell differentiation. Blood 108(6):1877-86. [PubMed: 16690964]  [MGI Ref ID J:138069]

Schmahl J; Raymond CS; Soriano P. 2007. PDGF signaling specificity is mediated through multiple immediate early genes. Nat Genet 39(1):52-60. [PubMed: 17143286]  [MGI Ref ID J:117491]

Silva RL; Thornton JD; Martin AC; Rehg JE; Bertwistle D; Zindy F; Skapek SX. 2005. Arf-dependent regulation of Pdgf signaling in perivascular cells in the developing mouse eye. EMBO J 24(15):2803-14. [PubMed: 16037818]  [MGI Ref ID J:101016]

Van den Akker NM; Winkel LC; Nisancioglu MH; Maas S; Wisse LJ; Armulik A; Poelmann RE; Lie-Venema H; Betsholtz C; Gittenberger-de Groot AC. 2008. PDGF-B signaling is important for murine cardiac development: Its role in developing atrioventricular valves, coronaries, and cardiac innervation. Dev Dyn 237(2):494-503. [PubMed: 18213589]  [MGI Ref ID J:130159]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX12

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, these mice can be bred as heterozygotes. Homozygotes have a perinatal lethal phenotype.
Diet Information LabDiet® 5K52/5K67

Purchasing information

Pricing, Supply Level & Notes, Controls, General Terms & Conditions

Pricing

Pricing for USA, Canada and Mexico shipping destinations             View   International   Pricing
Weeks of AgePrice*GenderGenotypes Provided
Individual Mouse Price $236.40Female or MaleHeterozygous for Pdgfrbtm1Sor
Pairs /Price*Pair Genotype
$288.65Heterozygous for Pdgfrbtm1Sor x Wild-type for Pdgfrbtm1Sor
$288.65Wild-type for Pdgfrbtm1Sor x Heterozygous for Pdgfrbtm1Sor
*Price(s) in US dollars ($)

Supply Details

Standard SupplyRepository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of ~nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within 48 hours of order placement.
Supply Notes

Pricing for International shipping destinations             View   USA, Canada and Mexico   Pricing
Weeks of AgePrice*GenderGenotypes Provided
Individual Mouse Price $307.40Female or MaleHeterozygous for Pdgfrbtm1Sor
Pairs /Price*Pair Genotype
$375.30Heterozygous for Pdgfrbtm1Sor x Wild-type for Pdgfrbtm1Sor
$375.30Wild-type for Pdgfrbtm1Sor x Heterozygous for Pdgfrbtm1Sor
*Price(s) in US dollars ($)

Supply Details

Standard SupplyRepository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of ~nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within 48 hours of order placement.
Supply Notes

Control Information

  Control
   Wild-type from the colony
   002448 129S1/SvImJ
 
  Considerations for Choosing Controls
  USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains.
  International - Control Pricing Information for Genetically Engineered Mutant Strains.

General Terms and Conditions

View JAX® Mice & Services Conditions of Use.

The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
Ordering and Purchasing Information

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Contact Information
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Tel: 800.422.6423 or 207.288.5845
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