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Phenotypes Associated with This Genotype
Genotype
MGI:3770662
Allelic
Composition
Il2rgtm1Wjl/Y
Prkdcscid/Prkdcscid
Genetic
Background
NOD.Cg-Prkdcscid Il2rgtm1Wjl/Sz
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Il2rgtm1Wjl mutation (50 available); any Il2rg mutation (86 available)
Prkdcscid mutation (100 available); any Prkdc mutation (129 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 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

cellular
• mice do not survive doses above or equal to 400cGy

hematopoietic system
• thymus consists mostly of stromal cells with sporadic cyst structures
• slight decrease in packed cell volume at 10 weeks as compared to to Prkdcscid controls
• decrease in peripheral leukocytes at 10 weeks as compared to to Prkdcscid controls
• flow cytometric analysis indicates that spleen cells exhibit a reduction in B220+ IgK- immature B cells in comparison to Prkdcscid controls
• spleens are deficient in B220+ IgK+ B cells
• 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
• spleens are deficient in mature T cells
• spleens are deficient in mature T cells
• spleens do not have detectable follicles
• two fold reduction in nucleated spleen cell numbers in comparison to Prkdcscid controls
• no detectable immunoglobulin in mice at 394-426 days of age

immune system
• decrease in peripheral leukocytes at 10 weeks as compared to to Prkdcscid controls
• flow cytometric analysis indicates that spleen cells exhibit a reduction in B220+ IgK- immature B cells in comparison to Prkdcscid controls
• spleens are deficient in B220+ IgK+ B cells
• 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
• spleens are deficient in mature T cells
• spleens are deficient in mature T cells
• no detectable immunoglobulin in mice at 394-426 days of age
• lymph tissues are severely depleted of lymphoid cells
• thymus consists mostly of stromal cells with sporadic cyst structures
• spleens do not have detectable follicles
• two fold reduction in nucleated spleen cell numbers in comparison to Prkdcscid controls
• lymph nodes in the double mutant are markedly smaller than those of homozygous Prkdcscid mice
• lymph nodes are hypocellular
• mice support CD34+ human stem cell engraftment at much higher levels than Prkdcscid controls (J:109833)
• 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 (J:109833)
• irradiated mice engrafted with human hematopoietic stem cells (HSCs) show much greater engraftment of human hematopoietic cells at 12 weeks than control NOD.129S7(B6)-Rag1tm1Mom homozygotes (J:140388)
• percentages of human CD45+ cells engrafted in bone marrow are higher than in NOD.CB17-Prkdcscid mice (J:140388)
• levels of human CD45+ cell and CD3+ T cell engraftment are higher in spleens than in NOD.CB17-Prkdcscid mice (J:140388)
• CD4:CD8 human T cell ratios that are close to normal physiological values and high levels of human B cells are observed in spleens of mice compared to NOD.CB17-Prkdcscid mice (J:140388)
• engraftment of human lymphohematopoietic cells in blood and thymus, and peripheral blood mononuclear cells (PMBC) engraftment are higher than in NOD.CB17-Prkdcscid (J:140388)

endocrine/exocrine glands
• thymus consists mostly of stromal cells with sporadic cyst structures


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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last database update
08/06/2019
MGI 6.14
The Jackson Laboratory