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Strain Name:

STOCK Il2rgtm1Cgn/J

Stock Number:

002479

Availability:

Repository-Cryopreserved


General Terms and Conditions

Former Name      STOCK Il2rgtm1Cgn    (Changed: 15-DEC-04 )
Genes & Alleles   Il2rg;   Il2rgtm1Cgn;


Product Information

Strain Details

Type JAX® GEMM® Strain - Mutant Stock
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Targeted Mutation
Specieslaboratory mouse
Donating Investigator Klaus Rajewsky,   Harvard Medical School
GenerationB6?+B6N4F3

Strain Description
Mice homozygous for the Il2rgtm1Cgn targeted mutation are viable and fertile. Mutant mice have hypoplastic thymuses with a 10-fold reduction in the absolute number of lymphocytes. They have a limited number of mature splenic B and T cells, lack NK cells and Peyer's patches, and development of gut-associated intraepithelial lymphocytes is diminished. In vitro studies show defects in NK activity, IL4-directed Ig class switching of thymocytes, and thymocyte mitogenic responses.

Related Disease (OMIM) Terms

NOTCombined Immunodeficiency, X-Linked; CIDX
NOTSevere Combined Immunodeficiency, X-Linked; SCIDX1
Mammalian Phenotype Terms assigned by genotype

Il2rgtm1Cgn/Il2rgtm1Cgn

        involves: 129 * CB20
  • immune system phenotype
  • abnormal leukocyte morphology (MGI Ref ID J:22521)
    • abnormal alpha-beta intraepithelial T cell morphology (MGI Ref ID J:22521)
      • 12-fold reduction in number of T cells found in gut epithelium
    • abnormal class switch recombination (MGI Ref ID J:22521)
      • in vitro class switch using LPS and IL-4 fail to generate more IgG1-positive cells
    • abnormal thymocyte activation (MGI Ref ID J:22521)
      • thymocytes do not activate in response to Con A
    • absent NK cells (MGI Ref ID J:22521)
      • NK cells are not detected in the spleen
    • decreased T cell number (MGI Ref ID J:22521)
      • 3 fold reduction of mature alphabeta T cells in the spleen
      • reduction of T cells in lamina propia of gut is observed
    • decreased T cell proliferation (MGI Ref ID J:22521)
      • splenic T cells do not proliferate in response to Con A
    • decreased mature B cell number (MGI Ref ID J:22521)
      • 12-fold reduction in the spleen in 3-4 week old mice
      • further loss occurs with age
      • decreased plasma cell number (MGI Ref ID J:22521)
        • plasma cells in the gut are exceptionally rare
    • decreased pre-B cell number (MGI Ref ID J:22521)
      • 20-fold decrease in bone marrow though some cells are still present
  • absent Peyer's patches (MGI Ref ID J:22521)
    • Peyer's patchers are not observed
  • absent spleen germinal center (MGI Ref ID J:22521)
    • no germinal centers are observed
  • decreased IgE level (MGI Ref ID J:22521)
    • no IgE detected in the sera
  • decreased IgM level (MGI Ref ID J:22521)
    • 3-fold reduction in sera levels
  • decreased spleen white pulp amount (MGI Ref ID J:22521)
    • clusters of white pulp are diminished in number and size
  • impaired NK cell cytolysis (MGI Ref ID J:22521)
    • no splenic NK killing activity is observed against a NK-sensitive target cell line
  • spleen hypoplasia (MGI Ref ID J:22521)
    • 8-fold reduction in cellularity
  • thymus hypoplasia (MGI Ref ID J:22521)
    • 22-fold reduction in cellularity
  • hematopoietic system phenotype
  • abnormal hematopoietic system morphology/development (MGI Ref ID J:22521)
    • abnormal leukocyte morphology (MGI Ref ID J:22521)
      • abnormal alpha-beta intraepithelial T cell morphology (MGI Ref ID J:22521)
        • 12-fold reduction in number of T cells found in gut epithelium
      • abnormal class switch recombination (MGI Ref ID J:22521)
        • in vitro class switch using LPS and IL-4 fail to generate more IgG1-positive cells
      • abnormal thymocyte activation (MGI Ref ID J:22521)
        • thymocytes do not activate in response to Con A
      • absent NK cells (MGI Ref ID J:22521)
        • NK cells are not detected in the spleen
      • decreased T cell number (MGI Ref ID J:22521)
        • 3 fold reduction of mature alphabeta T cells in the spleen
        • reduction of T cells in lamina propia of gut is observed
      • decreased T cell proliferation (MGI Ref ID J:22521)
        • splenic T cells do not proliferate in response to Con A
      • decreased mature B cell number (MGI Ref ID J:22521)
        • 12-fold reduction in the spleen in 3-4 week old mice
        • further loss occurs with age
        • decreased plasma cell number (MGI Ref ID J:22521)
          • plasma cells in the gut are exceptionally rare
      • decreased pre-B cell number (MGI Ref ID J:22521)
        • 20-fold decrease in bone marrow though some cells are still present
    • absent spleen germinal center (MGI Ref ID J:22521)
      • no germinal centers are observed
    • decreased spleen white pulp amount (MGI Ref ID J:22521)
      • clusters of white pulp are diminished in number and size
    • spleen hypoplasia (MGI Ref ID J:22521)
      • 8-fold reduction in cellularity
    • thymus hypoplasia (MGI Ref ID J:22521)
      • 22-fold reduction in cellularity

Il2rgtm1Cgn/Y

        involves: 129 * CB20
  • immune system phenotype
  • abnormal leukocyte morphology (MGI Ref ID J:22521)
    • abnormal alpha-beta intraepithelial T cell morphology (MGI Ref ID J:22521)
      • 12-fold reduction in number of T cells found in gut epithelium
    • abnormal class switch recombination (MGI Ref ID J:22521)
      • in vitro class switch using LPS and IL-4 fail to generate more IgG1-positive cells
    • abnormal gamma-delta T cell differentiation (MGI Ref ID J:111821)
      • complete absence of gamma-delta T cells in adult thymus
      • few gamma-delta T cells found in fetal thymus
      • TCR-gamma rearrangements are severely reduced
    • abnormal thymocyte activation (MGI Ref ID J:22521)
      • thymocytes do not activate in response to Con A
    • absent NK cells (MGI Ref ID J:22521)
      • NK cells are not detected in the spleen
    • decreased T cell number (MGI Ref ID J:22521)
      • 3 fold reduction of mature alphabeta T cells in the spleen
      • reduction of T cells in lamina propia of gut is observed
      • absent gamma-delta T cells (MGI Ref ID J:111821)
        • no gamma-delta T cells are found in peripheral lymphnoid organs or skin
        • absent gamma-delta intraepithelial T cell (MGI Ref ID J:111821)
    • decreased T cell proliferation (MGI Ref ID J:22521)
      • splenic T cells do not proliferate in response to Con A
    • decreased mature B cell number (MGI Ref ID J:22521)
      • 12-fold reduction in the spleen in 3-4 week old mice
      • further loss occurs with age
      • decreased plasma cell number (MGI Ref ID J:22521)
        • plasma cells in the gut are exceptionally rare
    • decreased pre-B cell number (MGI Ref ID J:22521)
      • 20-fold decrease in bone marrow though some cells are still present
  • absent Peyer's patches (MGI Ref ID J:22521)
    • Peyer's patchers are not observed
  • absent spleen germinal center (MGI Ref ID J:22521)
    • no germinal centers are observed
  • decreased IgE level (MGI Ref ID J:22521)
    • no IgE detected in the sera
  • decreased IgM level (MGI Ref ID J:22521)
    • 3-fold reduction in sera levels
  • decreased spleen white pulp amount (MGI Ref ID J:22521)
    • clusters of white pulp are diminished in number and size
  • impaired NK cell cytolysis (MGI Ref ID J:22521)
    • no splenic NK killing activity is observed against a NK-sensitive target cell line
  • spleen hypoplasia (MGI Ref ID J:22521)
    • 8-fold reduction in cellularity
  • thymus hypoplasia (MGI Ref ID J:22521)
    • 22-fold reduction in cellularity
    • 10-fold less cellularity of thymus from fetal day E15.5 through birth
    • 20-fold less cellularity by six days of age
  • hematopoietic system phenotype
  • abnormal leukocyte morphology (MGI Ref ID J:22521)
    • abnormal alpha-beta intraepithelial T cell morphology (MGI Ref ID J:22521)
      • 12-fold reduction in number of T cells found in gut epithelium
    • abnormal class switch recombination (MGI Ref ID J:22521)
      • in vitro class switch using LPS and IL-4 fail to generate more IgG1-positive cells
    • abnormal gamma-delta T cell differentiation (MGI Ref ID J:111821)
      • complete absence of gamma-delta T cells in adult thymus
      • few gamma-delta T cells found in fetal thymus
      • TCR-gamma rearrangements are severely reduced
    • abnormal thymocyte activation (MGI Ref ID J:22521)
      • thymocytes do not activate in response to Con A
    • absent NK cells (MGI Ref ID J:22521)
      • NK cells are not detected in the spleen
    • decreased T cell number (MGI Ref ID J:22521)
      • 3 fold reduction of mature alphabeta T cells in the spleen
      • reduction of T cells in lamina propia of gut is observed
      • absent gamma-delta T cells (MGI Ref ID J:111821)
        • no gamma-delta T cells are found in peripheral lymphnoid organs or skin
        • absent gamma-delta intraepithelial T cell (MGI Ref ID J:111821)
    • decreased T cell proliferation (MGI Ref ID J:22521)
      • splenic T cells do not proliferate in response to Con A
    • decreased mature B cell number (MGI Ref ID J:22521)
      • 12-fold reduction in the spleen in 3-4 week old mice
      • further loss occurs with age
      • decreased plasma cell number (MGI Ref ID J:22521)
        • plasma cells in the gut are exceptionally rare
    • decreased pre-B cell number (MGI Ref ID J:22521)
      • 20-fold decrease in bone marrow though some cells are still present
  • absent spleen germinal center (MGI Ref ID J:22521)
    • no germinal centers are observed
  • decreased spleen white pulp amount (MGI Ref ID J:22521)
    • clusters of white pulp are diminished in number and size
  • spleen hypoplasia (MGI Ref ID J:22521)
    • 8-fold reduction in cellularity
  • thymus hypoplasia (MGI Ref ID J:22521)
    • 22-fold reduction in cellularity
    • 10-fold less cellularity of thymus from fetal day E15.5 through birth
    • 20-fold less cellularity by six days of age

Gene & Allele Details

Allele Symbol Il2rgtm1Cgn
Allele Name targeted mutation 1, University of Cologne
Common Name(s) IL-2Rgamma-; Il2rg-; cgamma-; gamma-; gammac-;
Mutation Made By James DiSanto,   University of Cologne
Strain of Origin129P2/OlaHsd
ES Cell Line NameE14.1
ES Cell Line Strain129P2/OlaHsd
Gene Symbol and Name Il2rg, interleukin 2 receptor, gamma chain
Chromosome X
Gene Common Name(s) Ab2-183; CD132; IMD4; SCIDX; SCIDX1; [g]c; common cytokine receptor gamma chain; common gamma chain; gamma C receptor; gamma(c);
Molecular Note Insertion of a neomycin resistance cassette flanked by lox P sites, followed by homologous recombination and cre-mediated deletion, resulted in the excision of exons 2 - 6 of the gene. These exons encode the extracellular and transmembrane domains of the protein. [MGI Ref ID J:22521]

Control Information

  Allele   Control
 Il2rgtm1Cgn  000664 C57BL/6J (approximate)
 
  Considerations for Choosing Controls

Genotyping Protocols

Il2rgtm1Cgn

Related Strains

Strains carrying other alleles of Il2rg
003174   B6.129S4-Il2rgtm1Wjl/J
003169   C.129S4-Il2rgtm1Wjl/J
005557   NOD.Cg-Prkdcscid Il2rgtm1Wjl/SzJ
View Strains carrying other alleles of Il2rg     (3 strains)

Research Applications

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

Il2rgtm1Cgn related

Cancer Research
Growth Factors/Receptors/Cytokines

Immunology and Inflammation Research
Growth Factors/Receptors/Cytokines

Research Tools
Immunology and Inflammation Research (NK Cell Deficiency)

References

Selected Reference(s)

DiSanto JP; Muller W; Guy-Grand D; Fischer A; Rajewsky K. 1995. Lymphoid development in mice with a targeted deletion of the interleukin 2 receptor gamma chain. Proc Natl Acad Sci U S A 92(2):377-81. [PubMed: 7831294]  [MGI Ref ID J:22521]

Additional References

Price and Supply Information

Strain Name: STOCK Il2rgtm1Cgn/J
Stock Number: 002479

Price Details

IMPORTANT NOTE: Prices are based on shipping destination. To view prices, select your shipping destination.

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Supply Details

Standard SupplyRepository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.
Supply Notes Cryorecovery - Standard.
The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery.

Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services: Tel: 1-800-422-6423 or 1-207-288-5845; Email: jaxservices@jax.org.
This strain is included in the Induced Mutant Resource Colony collection.

LicensingSee General Terms and Conditions below for Licensing and Use Restrictions  
Control InformationView Control Information in Strain Details.

General Terms and Conditions

View JAX® Mice & Services Conditions of Use.

Effective September 26, 2007: License Requirements for Strains using Cre-lox Technology only apply in Canada, see Licenses for Strains using Cre-lox Technology.

For additional Licensing and Use Restrictions view the link(s) below:
- Use of MICE by companies or for-profit entities requires a license prior to shipping.

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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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