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Strain Name:

NOD.Cg-Prkdcscid Il2rgtm1Wjl/SzJ

Stock Number:

005557

Availability:

Level 3

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Strain Common Names      NSG;      NOD-scid IL2Rgammanull;      NOD-scid IL2Rgnull;
Genes & Alleles   Il2rg;   Il2rgtm1Wjl;   Prkdc;   Prkdcscid;

Product Information

Strain Details

Type JAX® GEMM® Strain - Congenic
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Mutant Strain
Type JAX® GEMM® Strain - Spontaneous Mutation
Type JAX® GEMM® Strain - Targeted Mutation
Mating SystemHomozygote x Homozygote         (Female x Male)
Specieslaboratory mouse
Donating Investigator Leonard Shultz,   The Jackson Laboratory
H2 Haplotypeg7
GenerationN8F?+4pF2 (03-JAN-08)

Appearance
albino

Strain Description
The NOD.Cg-Prkdcscid Il2rgtm1Wjl/SzJ mice, commonly known as NOD scid gamma (NSG), do not express the Prkdc gene nor the X-linked Il2rg gene. NSG mice are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. Histological examination of lymphoid tissues reveals absence of lymphoid cells and some cystic structures in the thymus, an absence of follicles in the spleen and markedly diminished celluarity of lymph nodes. NSG mice are deficient in mature lymphocytes, serum Ig is not detectable and natural killer (NK) cell cytotoxic activity is extremely low. These mice are resistant to lymphoma development even after sublethal irradiation treatment. These mutant mice have been shown to readily support engraftment of human CD34+ hematopoietic stem cells and represent a superior, long-lived model suitable for studies employing xenotransplantation strategies. Please note that the NSG carries the true null interleukin-2 receptor gamma chain mutation and should not be confused with the "NOG" mouse which has a truncated interleukin-2 receptor gamma chain as described in: "Modulation of hematopoiesis in mice with a truncated mutant of the interleukin-2 receptor gamma chain" Ohbo K et al. Blood 1996. 87:956-67.

Strain Development
These double mutant mice were produced by breeding female NOD.CB17-Prkdcscid/J (Stock No. 001303) mice with male mice bearing the X-linked B6.129S4-Il2rgtm1Wjl/J allele (Stock No. 003174). The resulting male mice heterozygous for the Prkdcscid allele and hemizygous for the Il2rgtm1Wjl allele were crossed to female NOD.CB17-Prkdcscid/J (Stock No. 001303) mice for 8 generations. Heterozygotes were interbred to produce mice homozygous for the Prkdcscid allele and homozygous (females) or hemizygous (males) for the Il2rgtm1Wjl allele.

Mammalian Phenotype Terms assigned by genotype

Il2rgtm1Wjl/Il2rgtm1Wjl Prkdcscid/Prkdcscid

        NOD.Cg-Prkdcscid Il2rgtm1Wjl/Sz
  • immune system phenotype
  • abnormal lymph organ cellularity (MGI Ref ID J:109833)
    • lymph tissues are severely depleted of lymphoid cells
    • abnormal lymph node cellularity (MGI Ref ID J:109833)
      • lymph nodes are hypocellular
    • abnormal spleen cellularity (MGI Ref ID J:109833)
      • two fold reduction in nucleated spleen cell numbers in comparison to Prkdcscid controls
    • abnormal thymus cellularity (MGI Ref ID J:109833)
      • thymus consists mostly of stromal cells with sporadic cyst structures
  • abnormal response to transplant (MGI Ref ID J:109833)
    • mice support CD34+ human stem cell engraftment at much higher levels than Prkdcscid controls
    • human CD45+ cells comprise almost 50% of cells in the spleen, approximately 35% in bone marrow and thymus, and 6% in blood at 10 weeks post-engraftment
  • abnormal spleen white pulp morphology (MGI Ref ID J:109833)
    • spleens do not have detectable follicles
  • decreased immunoglobulin level (MGI Ref ID J:109833)
    • no detectable immunoglobulin in mice at 394-426 days of age
  • decreased leukocyte cell number (MGI Ref ID J:109833)
    • decrease in peripheral leukocytes at 10 weeks as compared to to Prkdcscid controls
    • decreased CD4-positive T cell number (MGI Ref ID J:109833)
      • spleens are deficient in mature T cells
    • decreased CD8-positive T cell number (MGI Ref ID J:109833)
      • spleens are deficient in mature T cells
    • decreased NK cell number (MGI Ref ID J:109833)
      • mice are deficient in cells expressing NK cell markers in comparison to Prkdcscid controls
      • spleen cells from poly(I:C) stimulated 6-8 week old mice exhibit very low levels of NK cell cytotoxic activity
    • decreased immature B cell number (MGI Ref ID J:109833)
      • flow cytometric analysis indicates that spleen cells exhibit a reduction in B220+ IgK- immature B cells in comparison to Prkdcscid controls
    • decreased mature B cell number (MGI Ref ID J:109833)
      • spleens are deficient in B220+ IgK+ B cells
  • small lymph nodes (MGI Ref ID J:109833)
    • lymph nodes in the double mutant are markedly smaller than those of homozygous Prkdcscid mice
  • hematopoietic system phenotype
  • abnormal spleen cellularity (MGI Ref ID J:109833)
    • two fold reduction in nucleated spleen cell numbers in comparison to Prkdcscid controls
  • abnormal spleen white pulp morphology (MGI Ref ID J:109833)
    • spleens do not have detectable follicles
  • abnormal thymus cellularity (MGI Ref ID J:109833)
    • thymus consists mostly of stromal cells with sporadic cyst structures
  • decreased leukocyte cell number (MGI Ref ID J:109833)
    • decrease in peripheral leukocytes at 10 weeks as compared to to Prkdcscid controls
    • decreased CD4-positive T cell number (MGI Ref ID J:109833)
      • spleens are deficient in mature T cells
    • decreased CD8-positive T cell number (MGI Ref ID J:109833)
      • spleens are deficient in mature T cells
    • decreased NK cell number (MGI Ref ID J:109833)
      • mice are deficient in cells expressing NK cell markers in comparison to Prkdcscid controls
      • spleen cells from poly(I:C) stimulated 6-8 week old mice exhibit very low levels of NK cell cytotoxic activity
    • decreased immature B cell number (MGI Ref ID J:109833)
      • flow cytometric analysis indicates that spleen cells exhibit a reduction in B220+ IgK- immature B cells in comparison to Prkdcscid controls
    • decreased mature B cell number (MGI Ref ID J:109833)
      • spleens are deficient in B220+ IgK+ B cells
  • decreased mean corpuscular volume (MGI Ref ID J:109833)
    • slight decrease in packed cell volume at 10 weeks as compared to to Prkdcscid controls
  • cellular phenotype
  • increased cellular sensitivity to X-ray irradiation (MGI Ref ID J:109833)
    • mice do not survive doses above or equal to 400cGy
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:109833)
    • median survival time is lengthened to between 59-95 weeks (median 89 weeks) in contrast to the shorter lifespan observed homozygous Prkdcscid controls
    • the majority of autopsies indicate no evidence of lymphomas
  • tumorigenesis
  • decreased incidence of ionizing radiation-induced tumors (MGI Ref ID J:109833)
    • mice irradiated with doses below 400cGy do not exhibit thymic lymphomas in contrast to homozygous Prkdcscid controls

Il2rgtm1Wjl/Y Prkdcscid/Prkdcscid

        NOD.Cg-Prkdcscid Il2rgtm1Wjl/Sz
  • cellular phenotype
  • increased cellular sensitivity to X-ray irradiation (MGI Ref ID J:109833)
    • mice do not survive doses above or equal to 400cGy
  • hematopoietic system phenotype
  • abnormal spleen cellularity (MGI Ref ID J:109833)
    • two fold reduction in nucleated spleen cell numbers in comparison to Prkdcscid controls
  • abnormal spleen white pulp morphology (MGI Ref ID J:109833)
    • spleens do not have detectable follicles
  • abnormal thymus cellularity (MGI Ref ID J:109833)
    • thymus consists mostly of stromal cells with sporadic cyst structures
  • decreased leukocyte cell number (MGI Ref ID J:109833)
    • decrease in peripheral leukocytes at 10 weeks as compared to to Prkdcscid controls
    • decreased CD4-positive T cell number (MGI Ref ID J:109833)
      • spleens are deficient in mature T cells
    • decreased CD8-positive T cell number (MGI Ref ID J:109833)
      • spleens are deficient in mature T cells
    • decreased NK cell number (MGI Ref ID J:109833)
      • spleen cells from poly(I:C) stimulated 6-8 week old mice exhibit very low levels of NK cell cytotoxic activity
      • mice are deficient in cells expressing NK cell markers in comparison to Prkdcscid controls
    • decreased immature B cell number (MGI Ref ID J:109833)
      • flow cytometric analysis indicates that spleen cells exhibit a reduction in B220+ IgK- immature B cells in comparison to Prkdcscid controls
    • decreased mature B cell number (MGI Ref ID J:109833)
      • spleens are deficient in B220+ IgK+ B cells
  • decreased mean corpuscular volume (MGI Ref ID J:109833)
    • slight decrease in packed cell volume at 10 weeks as compared to to Prkdcscid controls
  • immune system phenotype
  • abnormal lymph organ cellularity (MGI Ref ID J:109833)
    • lymph tissues are severely depleted of lymphoid cells
    • abnormal lymph node cellularity (MGI Ref ID J:109833)
      • lymph nodes are hypocellular
    • abnormal spleen cellularity (MGI Ref ID J:109833)
      • two fold reduction in nucleated spleen cell numbers in comparison to Prkdcscid controls
    • abnormal thymus cellularity (MGI Ref ID J:109833)
      • thymus consists mostly of stromal cells with sporadic cyst structures
  • abnormal response to transplant (MGI Ref ID J:109833)
    • mice support CD34+ human stem cell engraftment at much higher levels than Prkdcscid controls
    • human CD45+ cells comprise almost 50% of cells in the spleen, approximately 35% in bone marrow and thymus, and 6% in blood at 10 weeks post-engraftment
  • abnormal spleen white pulp morphology (MGI Ref ID J:109833)
    • spleens do not have detectable follicles
  • decreased immunoglobulin level (MGI Ref ID J:109833)
    • no detectable immunoglobulin in mice at 394-426 days of age
  • decreased leukocyte cell number (MGI Ref ID J:109833)
    • decrease in peripheral leukocytes at 10 weeks as compared to to Prkdcscid controls
    • decreased CD4-positive T cell number (MGI Ref ID J:109833)
      • spleens are deficient in mature T cells
    • decreased CD8-positive T cell number (MGI Ref ID J:109833)
      • spleens are deficient in mature T cells
    • decreased NK cell number (MGI Ref ID J:109833)
      • spleen cells from poly(I:C) stimulated 6-8 week old mice exhibit very low levels of NK cell cytotoxic activity
      • mice are deficient in cells expressing NK cell markers in comparison to Prkdcscid controls
    • decreased immature B cell number (MGI Ref ID J:109833)
      • flow cytometric analysis indicates that spleen cells exhibit a reduction in B220+ IgK- immature B cells in comparison to Prkdcscid controls
    • decreased mature B cell number (MGI Ref ID J:109833)
      • spleens are deficient in B220+ IgK+ B cells
  • small lymph nodes (MGI Ref ID J:109833)
    • lymph nodes in the double mutant are markedly smaller than those of homozygous Prkdcscid mice
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:109833)
    • the majority of autopsies indicate no evidence of lymphomas
    • median survival time is lengthened to between 59-95 weeks (median 89 weeks) in contrast to the shorter lifespan observed homozygous Prkdcscid controls
  • tumorigenesis
  • decreased incidence of ionizing radiation-induced tumors (MGI Ref ID J:109833)
    • mice irradiated with doses below 400cGy do not exhibit thymic lymphomas in contrast to homozygous Prkdcscid controls

Gene & Allele Details

Allele Symbol Il2rgtm1Wjl
Allele Name targeted mutation 1, Warren J Leonard
Common Name(s) CD132-; IL2Rgammanull; [KO]gammac; gammac-;
Mutation Made By Warren Leonard,   NHLBI, NIH
Strain of Origin129S4/SvJae
ES Cell Line NameJ1
ES Cell Line Strain129S4/SvJae
Gene Symbol and Name Il2rg, interleukin 2 receptor, gamma chain
Chromosome X
Gene Common Name(s) CD132; IMD4; SCIDX; SCIDX1; [g]c; common cytokine receptor gamma chain; common gamma chain; gamma C receptor; gamma(c);
Molecular Note A neomycin resistance cassette replaced part of exon 3 and all of exons 4 - 8 of the gene, resulting in the loss of most of the extracellular domain and all of the transmembrane and cytoplasmic domains of the protein. [MGI Ref ID J:24117]
 
Allele Symbol Prkdcscid
Allele Name severe combined immunodeficiency
Common Name(s) scid;
Strain of OriginCB17
Gene Symbol and Name Prkdc, protein kinase, DNA activated, catalytic polypeptide
Chromosome 16
Gene Common Name(s) AI326420; AU019811; DNA-PK; DNA-PKcs; DNAPDcs; DNAPK; DNPK1; HYRC; HYRC1; XRCC7; expressed sequence AI326420; expressed sequence AU019811; p350; scid; severe combined immunodeficiency; slip;
General Note The Prkdcscid mutation arose in the C.B-17 inbred strain (BALB/c.C57BL/Ka-Igh-1b) (J:9341). Most homozygotes have no detectable IgM, IgG1, IgG2a, IgG2b, IgG3, or IgA, but a few have low levels of one to three of these immunoglobulin isotypes. The size of the lymphoid organs is only one-tenth or less that of normal. Thymus, lymph nodes, and splenic follicles are virtually devoid of lymphocytes (J:30980).

Homozygotes are deficient in both B and T cell function. Their spleen cells do not respond to either B or T cell mitogens and they are unable to reject skin grafts. They lack detectable B cells and pre-B cells. In spite of the small thymus and lack of functional T cells, the Thy1 marker is present on a majority of cells recovered from the thymus, and T cell lymphomas occur in 10 per cent or more of affected mice. Prkdcscid specifically impairs differentiation of stem cells into mature lymphocytes. Myeloid cell differentiation is not affected. The basic defect in these mice appears to be in the lymphoid stem cells and not in the cellular environment, since functional T and B cells are found in mice reconstituted with normal bone marrow (J:30980, J:7343). However, full reconstitution of the immune deficiency occurs only after irradiation of the recipients, indicating that Prkdcscid/Prkdcscid mice may have normal numbers of a radiation-sensitive stem cell that has defective proliferative capacity (J:8299).

The rearrangements of immunoglobulin and T cell receptor genes that normally occur in B and T lymphocytes are not found in homozygous Prkdcscid mice. However, in Abelson leukemia virus-transformed B cells of these mice and in their occasional T cell lymphomas, rearrangements, most of which are abnormal, are found. This suggests that scid may act through an effect on the recombinase system catalyzing the assembly of immunoglobulin and T cell receptor genes, and that lymphocytes with these defects are not able to develop further (J:8420).

Although most Prkdcscid homozygotes fail to produce immunoglobulin and functional T-cell receptor, some produce these products at low levels, with an occasional mouse with nearly normal levels of serum immunoglobulin, the criterion usually used tomeasure the effects of Prkdcscid. This phenomenon is referred to as "leakiness" of the VDJ recombination defect (J:4610).Homozygous Prkdcscidmice are fertile and, under specific pathogen-free conditions, may survive a year or more(J:6958).

The Prkdcscid mouse has been widely used in studies of the immune system, in particular of VDJ recombination in T and B lymphocytes. Its lack of immunocompetence has made it useful in transplantation studies, particularly transplantation and development of metastasis in human tumors. The interaction of infection, immunity, and disease processes have been studied with these mice. Poole (J:31292) offers a brief review of the nature and usefulness of the Prkdcscid mouse, with key references to the very extensive literature.

Mutant mRNA does not appear to differ from wild type although protein expression is reduced more than 10-fold. Mutant protein is defective for nuclear association but exhibits normal DNA-binding ability.

NOD.Cg-Prkdcscid B2mtm1Unc mice lack mature lymphocytes and serum Ig, are MHC class I deficient, B and T cell deficient, C-5 deficient (Hc0), and have low NK cells. These mice display accumulation of iron in the liver and rapid clearance of human IgG1.

Molecular Note A T-to-A transversion point mutation at a position corresponding to codon 4095 created a premature stop codon. [MGI Ref ID J:35393] [MGI Ref ID J:39329]

Control Information

  Control
   001303 NOD.CB17-Prkdcscid/J
   001976 NOD/ShiLtJ
 
  Considerations for Choosing Controls
  Control Pricing Information for JAX® GEMM® Strains

Genotyping Protocols

Il2rgtm1Wjl
Prkdcscid

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, these mice are bred as homozygotes for both the Prkdcscid and Il2rgtm1Wjl alleles. SPF (specific pathogen-free) conditions are recommended.
Diet Information LabDiet® 5K52/5K67

Related Strains

Strains carrying   Il2rgtm1Wjl allele
003174   B6.129S4-Il2rgtm1Wjl/J
003169   C.129S4-Il2rgtm1Wjl/J
007799   NOD.Cg-Rag1tm1Mom Il2rgtm1Wjl/SzJ
View Strains carrying   Il2rgtm1Wjl     (3 strains)

View Strains carrying   Prkdcscid     (25 strains)

Strains carrying other alleles of Il2rg
002479   STOCK Il2rgtm1Cgn/J
View Strains carrying other alleles of Il2rg     (1 strain)

Additional Web Information

Genetic Quality Control Annual Report
JAX® NOTES, Spring 2006; 501. Choosing an Immunodeficient Mouse Model.
JAX® NOTES, Spring 2008; 509. Jackson Laboratory's Leonard Shultz PhD Helps Develop a Better Leukemia Mouse Model.
JAX® NOTES, Summer 2005; 498. NOD.Cg-Prkdcscid Il2rgtm1Wjl/Sz, a New Model for Engraftment with Human Hematopoietic Stem Cells.
Strain-at-a-glance.

Animal Health Reports

Room Number           AX30
Room Number           RB08

Research Applications

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

Research Tools
Immunology and Inflammation Research (B, T, and NK cell deficiency)

Il2rgtm1Wjl related

Cancer Research
Growth Factors/Receptors/Cytokines

Immunology and Inflammation Research
Growth Factors/Receptors/Cytokines

Prkdcscid related
Immunodeficiency (B and T cell deficiency)

Internal/Organ Research
Lymphoid Tissue Defects (B and T cell deficiency)

Research Tools
Cancer Research (B and T cell deficiency) (xenograft/transplant host)
Toxicology Research (xenograft/transplant host)

Virology Research
B and T Cell Deficiency (AIDS research tool)

References

Selected Reference(s)

Shultz LD; Lyons BL; Burzenski LM; Gott B; Chen X; Chaleff S; Kotb M; Gillies SD; King M; Mangada J; Greiner DL; Handgretinger R. 2005. Human lymphoid and myeloid cell development in NOD/LtSz-scid IL2R gamma null mice engrafted with mobilized human hemopoietic stem cells. J Immunol 174(10):6477-89. [PubMed: 15879151]  [MGI Ref ID J:109833]

Additional References

Price and Supply Information

Strain Name: NOD.Cg-Prkdcscid Il2rgtm1Wjl/SzJ
Stock Number: 005557

Price Details

IMPORTANT NOTE: Prices are based on shipping destination. The shipping destinations are:

*Pricing for Shipping Destination selected:

        International

Price(s) in US dollars ($)Genotype(s) Provided
3-5 weeks $107.20Homozygous for Prkdcscid, Homozygous for Il2rgtm1WjlFemale
6 weeks $112.30Homozygous for Prkdcscid, Homozygous for Il2rgtm1WjlFemale
7 weeks $117.40Homozygous for Prkdcscid, Homozygous for Il2rgtm1WjlFemale
8 weeks $122.50Homozygous for Prkdcscid, Homozygous for Il2rgtm1WjlFemale
9 weeks $127.60Homozygous for Prkdcscid, Homozygous for Il2rgtm1WjlFemale
10 weeks $132.70Homozygous for Prkdcscid, Homozygous for Il2rgtm1WjlFemale
11 weeks $137.80Homozygous for Prkdcscid, Homozygous for Il2rgtm1WjlFemale
12 weeks $142.90Homozygous for Prkdcscid, Homozygous for Il2rgtm1WjlFemale
13 weeks $148.00Homozygous for Prkdcscid, Homozygous for Il2rgtm1WjlFemale
14 weeks $153.10Homozygous for Prkdcscid, Homozygous for Il2rgtm1WjlFemale
15 weeks $158.20Homozygous for Prkdcscid, Homozygous for Il2rgtm1WjlFemale
3-5 weeks $107.20Homozygous for Prkdcscid, Hemizygous for Il2rgtm1WjlMale
6 weeks $112.30Homozygous for Prkdcscid, Hemizygous for Il2rgtm1WjlMale
7 weeks $117.40Homozygous for Prkdcscid, Hemizygous for Il2rgtm1WjlMale
8 weeks $122.50Homozygous for Prkdcscid, Hemizygous for Il2rgtm1WjlMale
9 weeks $127.60Homozygous for Prkdcscid, Hemizygous for Il2rgtm1WjlMale
10 weeks $132.70Homozygous for Prkdcscid, Hemizygous for Il2rgtm1WjlMale
11 weeks $137.80Homozygous for Prkdcscid, Hemizygous for Il2rgtm1WjlMale
12 weeks $142.90Homozygous for Prkdcscid, Hemizygous for Il2rgtm1WjlMale
13 weeks $148.00Homozygous for Prkdcscid, Hemizygous for Il2rgtm1WjlMale
14 weeks $153.10Homozygous for Prkdcscid, Hemizygous for Il2rgtm1WjlMale
15 weeks $158.20Homozygous for Prkdcscid, Hemizygous for Il2rgtm1WjlMale
Pair $224.40Homozygous for Prkdcscid, Homozygous for Il2rgtm1Wjl x Homozygous for Prkdcscid, Hemizygous for Il2rgtm1Wjl

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Standard SupplyLevel 3. Up to 50 mice. Larger quantities or custom orders arranged upon request.
Supply Notes Histology and Tissue Collection Services are available for all JAX® Mice strains. For more information, please contact Customer Service at orderquest@jax.org or 1-207-288-5845.
Shipped at a specific age in weeks. Mice at a precise age in days, littermates and retired breeders are also available.
Strains that must be genotyped are not available until five to seven weeks of age.
This strain is included in the Induced Mutant Resource Colony collection.
Genomic DNA is available for this strain from the Mouse DNA Resource.
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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.
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