Phenotypes associated with this allele

• Allele Symbol: Il2rg^tm1Wjl
• Allele Name: targeted mutation 1, Warren J Leonard
• Allele ID: MGI:1857455
• Summary: 13 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Il2rg^tm1Wjl/Il2rg^tm1Wjl	B6.129S4-Il2rg^tm1Wjl/J	MGI:3769353
cn2	B2m^tm1Unc/B2m^tm1Unc Il2rg^tm1.1Asin/Il2rg^tm1Wjl Tg(Cd8a*-cre)B8Asin/0	involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6	MGI:5469014
cn3	Il2rg^tm1.1Asin/Il2rg^tm1Wjl Tg(Cd8a*-cre)B8Asin/0	involves: 129S4/SvJae * C57BL/6	MGI:5469012
cn4	Il2rg^tm1.1Asin/Il2rg^tm1Wjl Tg(Cd8a*-cre)B8Asin/0 Tg(LCKprBCL2)36Sjk/0	involves: 129S4/SvJae * C57BL/6	MGI:5469013
cn5	Il2rg^tm1.1Asin/Il2rg^tm1Wjl Tg(Cd4-cre)1Cwi/0	involves: 129S4/SvJae * C57BL/6 * DBA/2	MGI:5469011
cx6	Il2rg^tm1Wjl/Il2rg^tm1Wjl Tg(CD2-Ccdc86)1Hfuj/?	B6.Cg-Il2rg^tm1Wjl Tg(CD2-Ccdc86)1Hfuj	MGI:4356292
cx7	Il2rg^tm1Wjl/Y Crlf2^tm1Wjl/Crlf2^tm1Wjl	involves: 129S4/SvJae * C57BL/6	MGI:3624431
cx8	Il2rg^tm1Wjl/Il2rg^tm1Wjl Rag2^tm1Fwa/Rag2^tm1Fwa	involves: 129S/SvEv * 129S4/SvJae	MGI:3817878
cx9	Del(7Pira1-Lilra6)1Fgla/Del(7Pira1-Lilra6)1Fgla Il2rg^tm1Wjl/Il2rg^tm1Wjl Rag2^tm1Fwa/Rag2^tm1Fwa	involves: C57BL/6J * C57BL/6NTac	MGI:6694871
cx10	Il2rg^tm1Wjl/Y Prkdc^scid/Prkdc^scid	NOD.Cg-Prkdc^scid Il2rg^tm1Wjl/Sz	MGI:3770662
cx11	Il2rg^tm1Wjl/Il2rg^tm1Wjl Prkdc^scid/Prkdc^scid	NOD.Cg-Prkdc^scid Il2rg^tm1Wjl/Sz	MGI:3770657
cx12	Il2rg^tm1Wjl/Y Rag1^tm1Mom/Rag1^tm1Mom	NOD.Cg-Rag1^tm1Mom Il2rg^tm1Wjl/SzJ	MGI:3813939
ot13	Il2rg^tm1Wjl/Y	involves: 129S4/SvJae * C57BL/6	MGI:3624447

Genotype
MGI:3769353

hm1

• Allelic Composition: Il2rg^tm1Wjl/Il2rg^tm1Wjl
• Genetic Background: B6.129S4-Il2rg^tm1Wjl/J

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

immune system

decreased myeloid dendritic cell number ( J:123902 )

♀ • the proportion and absolute number of CD11c+ dendritic cells is decreased in the spleen

abnormal splenic cell ratio ( J:123902 )

♀ • reduced numbers of splenocytes

♀ • decrease in the proportion and absolute number of CD11c+ dendritic cells in the spleen

abnormal dendritic cell physiology ( J:123902 )

♀ • in culture, dendritic cells are resistant to cytokine withdrawal-induced apoptosis

♀ • bone marrow-derived dendritic cells overproduce IL12 and IL10

hematopoietic system

decreased myeloid dendritic cell number ( J:123902 )

♀ • the proportion and absolute number of CD11c+ dendritic cells is decreased in the spleen

abnormal splenic cell ratio ( J:123902 )

♀ • reduced numbers of splenocytes

♀ • decrease in the proportion and absolute number of CD11c+ dendritic cells in the spleen

Genotype
MGI:5469014

cn2

• Allelic Composition: B2m^tm1Unc/B2m^tm1Unc; Il2rg^tm1.1Asin/Il2rg^tm1Wjl; Tg(Cd8a*-cre)B8Asin/0
• Genetic Background: involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6

immune system

decreased CD8-positive, alpha-beta T cell number ( J:190889 )

• in the thymus

hematopoietic system

decreased CD8-positive, alpha-beta T cell number ( J:190889 )

• in the thymus

Genotype
MGI:5469012

cn3

• Allelic Composition: Il2rg^tm1.1Asin/Il2rg^tm1Wjl; Tg(Cd8a*-cre)B8Asin/0
• Genetic Background: involves: 129S4/SvJae * C57BL/6

immune system

decreased CD4-positive, alpha-beta T cell number ( J:190889 )

• in the thymus

decreased CD8-positive, alpha-beta T cell number ( J:190889 )

• in the thymus but not the periphery due to lymphopenia-induced homeostatic expansion

decreased NK T cell number ( J:190889 )

• of invariant NK T cells in the thymus, spleen and liver

decreased regulatory T cell number ( J:190889 )

• of CD4 single-positive thymocytes expressing Foxp3

• however, these cells are restored by a cross with Tg(LCKprBCL2)36Sjk mice

hematopoietic system

decreased CD4-positive, alpha-beta T cell number ( J:190889 )

• in the thymus

decreased CD8-positive, alpha-beta T cell number ( J:190889 )

• in the thymus but not the periphery due to lymphopenia-induced homeostatic expansion

decreased NK T cell number ( J:190889 )

• of invariant NK T cells in the thymus, spleen and liver

decreased regulatory T cell number ( J:190889 )

• of CD4 single-positive thymocytes expressing Foxp3

• however, these cells are restored by a cross with Tg(LCKprBCL2)36Sjk mice

Genotype
MGI:5469013

cn4

• Allelic Composition: Il2rg^tm1.1Asin/Il2rg^tm1Wjl; Tg(Cd8a*-cre)B8Asin/0; Tg(LCKprBCL2)36Sjk/0
• Genetic Background: involves: 129S4/SvJae * C57BL/6

immune system

decreased NK T cell number ( J:190889 )

• of invariant NK T cells as in Il2rg^tm1.1Asin/Il2rg^tm1Wjl Tg(Cd8a*-cre)#Asin mice

hematopoietic system

decreased NK T cell number ( J:190889 )

• of invariant NK T cells as in Il2rg^tm1.1Asin/Il2rg^tm1Wjl Tg(Cd8a*-cre)#Asin mice

Genotype
MGI:5469011

cn5

• Allelic Composition: Il2rg^tm1.1Asin/Il2rg^tm1Wjl; Tg(Cd4-cre)1Cwi/0
• Genetic Background: involves: 129S4/SvJae * C57BL/6 * DBA/2

immune system

decreased CD4-positive, alpha-beta T cell number ( J:190889 )

• in the thymus

decreased CD8-positive, alpha-beta T cell number ( J:190889 )

• in the thymus

hematopoietic system

decreased CD4-positive, alpha-beta T cell number ( J:190889 )

• in the thymus

decreased CD8-positive, alpha-beta T cell number ( J:190889 )

• in the thymus

Genotype
MGI:4356292

cx6

• Allelic Composition: Il2rg^tm1Wjl/Il2rg^tm1Wjl; Tg(CD2-Ccdc86)1Hfuj/?
• Genetic Background: B6.Cg-Il2rg^tm1Wjl Tg(CD2-Ccdc86)1Hfuj

immune system

immune system phenotype ( J:151722 )

♀N • the presence of the transgene prevents the splenomegaly associated with Il2rg^tm1Wjl homozygotes due to decreased lymphocyte numbers and size

decreased CD4-positive, alpha-beta T cell number ( J:151722 )

♀ • CD4 T cell numbers are decreased compared to wild-type and to Il2rg^tm1Wjl homozygotes

decreased CD8-positive, alpha-beta T cell number ( J:151722 )

♀ • CD8 T cell numbers are decreased compared to wild-type and to Il2rg^tm1Wjl homozygotes

decreased regulatory T cell number ( J:151722 )

♀ • the transgene fails to rescue the decreased numbers of FoxP3⁺ regulatory T cells associated with Il2rg^tm1Wjl homozygotes

decreased splenocyte number ( J:151722 )

♀ • splenocyte number is decreased compared to wild-type and to Il2rg^tm1Wjl homozygotes

hematopoietic system

decreased CD4-positive, alpha-beta T cell number ( J:151722 )

♀ • CD4 T cell numbers are decreased compared to wild-type and to Il2rg^tm1Wjl homozygotes

decreased CD8-positive, alpha-beta T cell number ( J:151722 )

♀ • CD8 T cell numbers are decreased compared to wild-type and to Il2rg^tm1Wjl homozygotes

decreased regulatory T cell number ( J:151722 )

♀ • the transgene fails to rescue the decreased numbers of FoxP3⁺ regulatory T cells associated with Il2rg^tm1Wjl homozygotes

decreased splenocyte number ( J:151722 )

♀ • splenocyte number is decreased compared to wild-type and to Il2rg^tm1Wjl homozygotes

Genotype
MGI:3624431

cx7

• Allelic Composition: Il2rg^tm1Wjl/Y; Crlf2^tm1Wjl/Crlf2^tm1Wjl
• Genetic Background: involves: 129S4/SvJae * C57BL/6

immune system

thymus hypoplasia ( J:108209 )

♂ • double knockout mice have very reduced thymic cellularity; cellularity is reduced from that observed in Ilr2g single knockouts which is significantly less than that of controls

hematopoietic system

thymus hypoplasia ( J:108209 )

♂ • double knockout mice have very reduced thymic cellularity; cellularity is reduced from that observed in Ilr2g single knockouts which is significantly less than that of controls

endocrine/exocrine glands

thymus hypoplasia ( J:108209 )

♂ • double knockout mice have very reduced thymic cellularity; cellularity is reduced from that observed in Ilr2g single knockouts which is significantly less than that of controls

Genotype
MGI:3817878

cx8

• Allelic Composition: Il2rg^tm1Wjl/Il2rg^tm1Wjl; Rag2^tm1Fwa/Rag2^tm1Fwa
• Genetic Background: involves: 129S/SvEv * 129S4/SvJae

mortality/aging

increased susceptibility to induced morbidity/mortality ( J:140483 )

♀ • animals succumb to M. tuberculosis within 30 days, showing same median survival time as Ifng-null mice while wild-type survive infection for entire period of observation

immune system

absent NK cells ( J:140483 )

♀

absent T cells ( J:140483 )

♀

abnormal interferon secretion ( J:140483 )

♀ • cells do not produce any interferon gamma after incubation of splenocytes with mycobacteria

lung inflammation ( J:140483 )

♀ • at 28 days post-infection (p.i.), lungs display massive lesions occupying about 60% of lung space compared to lungs in Rag2-null mice

increased susceptibility to bacterial infection ( J:140483 )

♀ • mice are more susceptible to infection than Rag2-null single mutants and show similar survival time to Ifng-null mice

♀ • in lungs and spleens of M. tuberculosis-infected mice at 28 days post-infection (p.i.), bacterial loads are >2 log higher than in infected wild-type mice

respiratory system

lung inflammation ( J:140483 )

♀ • at 28 days post-infection (p.i.), lungs display massive lesions occupying about 60% of lung space compared to lungs in Rag2-null mice

hematopoietic system

absent NK cells ( J:140483 )

♀

absent T cells ( J:140483 )

♀

Genotype
MGI:6694871

cx9

• Allelic Composition: Del(7Pira1-Lilra6)1Fgla/Del(7Pira1-Lilra6)1Fgla; Il2rg^tm1Wjl/Il2rg^tm1Wjl; Rag2^tm1Fwa/Rag2^tm1Fwa
• Genetic Background: involves: C57BL/6J * C57BL/6NTac

immune system

abnormal response to transplant ( J:290663 )

• no monocyte memory response to BALB/c allograft 7 or 28 days after priming with irradiated BALB/c splenocytes

• normal primary response (primary dendritic cell and macrophage functions) to BALB/c allograft after priming with irradiated BALB/c splenocytes

Genotype
MGI:3770662

cx10

• Allelic Composition: Il2rg^tm1Wjl/Y; Prkdc^scid/Prkdc^scid
• Genetic Background: NOD.Cg-Prkdc^scid Il2rg^tm1Wjl/Sz

mortality/aging

premature death ( 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 Prkdc^scid controls

cellular

increased cellular sensitivity to X-ray irradiation ( J:109833 )

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

hematopoietic system

abnormal thymus morphology ( J:109833 )

♂ • thymus consists mostly of stromal cells with sporadic cyst structures

decreased mean corpuscular volume ( J:109833 )

♂ • slight decrease in packed cell volume at 10 weeks as compared to to Prkdc^scid controls

decreased leukocyte cell number ( J:109833 )

♂ • decrease in peripheral leukocytes at 10 weeks as compared to to Prkdc^scid controls

decreased immature B cell number ( J:109833 )

♂ • flow cytometric analysis indicates that spleen cells exhibit a reduction in B220+ IgK- immature B cells in comparison to Prkdc^scid controls

decreased mature B cell number ( J:109833 )

♂ • spleens are deficient in B220+ IgK+ B cells

decreased NK cell number ( 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 Prkdc^scid controls

decreased CD4-positive, alpha-beta T cell number ( J:109833 )

♂ • spleens are deficient in mature T cells

decreased CD8-positive, alpha-beta T cell number ( J:109833 )

♂ • spleens are deficient in mature T cells

decreased spleen B cell follicle number ( J:109833 )

♂ • spleens do not have detectable follicles

abnormal splenic cell ratio ( J:109833 )

♂ • two fold reduction in nucleated spleen cell numbers in comparison to Prkdc^scid controls

decreased immunoglobulin level ( J:109833 )

♂ • no detectable immunoglobulin in mice at 394-426 days of age

immune system

decreased leukocyte cell number ( J:109833 )

♂ • decrease in peripheral leukocytes at 10 weeks as compared to to Prkdc^scid controls

decreased immature B cell number ( J:109833 )

♂ • flow cytometric analysis indicates that spleen cells exhibit a reduction in B220+ IgK- immature B cells in comparison to Prkdc^scid controls

decreased mature B cell number ( J:109833 )

♂ • spleens are deficient in B220+ IgK+ B cells

decreased NK cell number ( 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 Prkdc^scid controls

decreased CD4-positive, alpha-beta T cell number ( J:109833 )

♂ • spleens are deficient in mature T cells

decreased CD8-positive, alpha-beta T cell number ( J:109833 )

♂ • spleens are deficient in mature T cells

decreased immunoglobulin level ( J:109833 )

♂ • no detectable immunoglobulin in mice at 394-426 days of age

abnormal immune system organ morphology ( J:109833 )

♂ • lymph tissues are severely depleted of lymphoid cells

abnormal thymus morphology ( J:109833 )

♂ • thymus consists mostly of stromal cells with sporadic cyst structures

decreased spleen B cell follicle number ( J:109833 )

♂ • spleens do not have detectable follicles

abnormal splenic cell ratio ( J:109833 )

♂ • two fold reduction in nucleated spleen cell numbers in comparison to Prkdc^scid controls

small lymph nodes ( J:109833 )

♂ • lymph nodes in the double mutant are markedly smaller than those of homozygous Prkdc^scid mice

lymph node hypoplasia ( J:109833 )

♂ • lymph nodes are hypocellular

abnormal response to transplant ( J:109833 , J:140388 )

♂ • mice support CD34+ human stem cell engraftment at much higher levels than Prkdc^scid 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)-Rag1^tm1Mom homozygotes (J:140388)

♂ • percentages of human CD45+ cells engrafted in bone marrow are higher than in NOD.CB17-Prkdc^scid mice (J:140388)

♂ • levels of human CD45+ cell and CD3+ T cell engraftment are higher in spleens than in NOD.CB17-Prkdc^scid 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-Prkdc^scid 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-Prkdc^scid (J:140388)

endocrine/exocrine glands

abnormal thymus morphology ( J:109833 )

♂ • thymus consists mostly of stromal cells with sporadic cyst structures

Genotype
MGI:3770657

cx11

• Allelic Composition: Il2rg^tm1Wjl/Il2rg^tm1Wjl; Prkdc^scid/Prkdc^scid
• Genetic Background: NOD.Cg-Prkdc^scid Il2rg^tm1Wjl/Sz

growth/size/body

thymus cyst ( J:109833 )

♀ • sporadic cyst structures

mortality/aging

premature death ( J:109833 )

♀ • median survival time is lengthened to between 59-95 weeks (median 89 weeks) in contrast to the shorter lifespan observed homozygous Prkdc^scid controls

♀ • the majority of autopsies indicate no evidence of lymphomas

immune system

decreased leukocyte cell number ( J:109833 )

♀ • decrease in peripheral leukocytes at 10 weeks as compared to to Prkdc^scid controls

decreased immature B cell number ( J:109833 )

♀ • flow cytometric analysis indicates that spleen cells exhibit a reduction in B220+ IgK- immature B cells in comparison to Prkdc^scid controls

decreased mature B cell number ( J:109833 )

♀ • spleens are deficient in B220+ IgK+ B cells

decreased NK cell number ( J:109833 )

♀ • mice are deficient in cells expressing NK cell markers in comparison to Prkdc^scid controls

♀ • spleen cells from poly(I:C) stimulated 6-8 week old mice exhibit very low levels of NK cell cytotoxic activity

decreased CD4-positive, alpha-beta T cell number ( J:109833 )

♀ • spleens are deficient in mature T cells

decreased CD8-positive, alpha-beta T cell number ( J:109833 )

♀ • spleens are deficient in mature T cells

decreased immunoglobulin level ( J:109833 )

♀ • no detectable immunoglobulin in mice at 394-426 days of age

abnormal immune system organ morphology ( J:109833 )

♀ • lymph tissues are severely depleted of lymphoid cells

abnormal thymus morphology ( J:109833 )

♀ • thymus consists mostly of stromal cells with sporadic cyst structures

thymus cyst ( J:109833 )

♀ • sporadic cyst structures

decreased spleen B cell follicle number ( J:109833 )

♀ • spleens do not have detectable follicles

abnormal splenic cell ratio ( J:109833 )

♀ • two fold reduction in nucleated spleen cell numbers in comparison to Prkdc^scid controls

small lymph nodes ( J:109833 )

♀ • lymph nodes in the double mutant are markedly smaller than those of homozygous Prkdc^scid mice

lymph node hypoplasia ( J:109833 )

♀ • lymph nodes are hypocellular

abnormal response to transplant ( J:109833 )

♀ • mice support CD34+ human stem cell engraftment at much higher levels than Prkdc^scid 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

hematopoietic system

abnormal thymus morphology ( J:109833 )

♀ • thymus consists mostly of stromal cells with sporadic cyst structures

thymus cyst ( J:109833 )

♀ • sporadic cyst structures

decreased mean corpuscular volume ( J:109833 )

♀ • slight decrease in packed cell volume at 10 weeks as compared to to Prkdc^scid controls

decreased leukocyte cell number ( J:109833 )

♀ • decrease in peripheral leukocytes at 10 weeks as compared to to Prkdc^scid controls

decreased immature B cell number ( J:109833 )

♀ • flow cytometric analysis indicates that spleen cells exhibit a reduction in B220+ IgK- immature B cells in comparison to Prkdc^scid controls

decreased mature B cell number ( J:109833 )

♀ • spleens are deficient in B220+ IgK+ B cells

decreased NK cell number ( J:109833 )

♀ • mice are deficient in cells expressing NK cell markers in comparison to Prkdc^scid controls

♀ • spleen cells from poly(I:C) stimulated 6-8 week old mice exhibit very low levels of NK cell cytotoxic activity

decreased CD4-positive, alpha-beta T cell number ( J:109833 )

♀ • spleens are deficient in mature T cells

decreased CD8-positive, alpha-beta T cell number ( J:109833 )

♀ • spleens are deficient in mature T cells

decreased spleen B cell follicle number ( J:109833 )

♀ • spleens do not have detectable follicles

abnormal splenic cell ratio ( J:109833 )

♀ • two fold reduction in nucleated spleen cell numbers in comparison to Prkdc^scid controls

decreased immunoglobulin level ( J:109833 )

♀ • no detectable immunoglobulin in mice at 394-426 days of age

cellular

increased cellular sensitivity to X-ray irradiation ( J:109833 )

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

neoplasm

decreased incidence of tumors by ionizing radiation induction ( J:109833 )

♀ • mice irradiated with doses below 400cGy do not exhibit thymic lymphomas in contrast to homozygous Prkdc^scid controls

homeostasis/metabolism

abnormal glucose homeostasis ( J:138005 )

♀ • streptozotocin-diabetic mice are not restored to euglycemia after receiving transplants of 1000 to 3000 IEQ, while 4000 IEQ transplant was successful in two long-term survivors

hyperglycemia ( J:138005 )

♀ • streptozotocin effectively induced hyperglycemia essentially equally in male and female mice at doses of 120to 160 mg/kg; some mice do not become hyperglycemic at all doses of streptozotocin administered

endocrine/exocrine glands

abnormal thymus morphology ( J:109833 )

♀ • thymus consists mostly of stromal cells with sporadic cyst structures

thymus cyst ( J:109833 )

♀ • sporadic cyst structures

Genotype
MGI:3813939

cx12

• Allelic Composition: Il2rg^tm1Wjl/Y; Rag1^tm1Mom/Rag1^tm1Mom
• Genetic Background: NOD.Cg-Rag1^tm1Mom Il2rg^tm1Wjl/SzJ

growth/size/body

thymus cyst ( J:140388 )

♂ • thymus contains large cysts

immune system

abnormal thymus morphology ( J:140388 )

♂ • no lymphoid cells are present

absent thymus cortex ( J:140388 )

♂ • no defined cortical region is observed

absent thymus medulla ( J:140388 )

♂ • no defined medullary region is observed

thymus cyst ( J:140388 )

♂ • thymus contains large cysts

absent NK T cells ( J:140388 )

♂ • natural killer cell (NK; DX5+LGL+ cells) are absent

decreased macrophage cell number ( J:140388 )

♂ • levels are Gr-1-ve Mac-1+ve macrophages are reduced

abnormal spleen morphology ( J:140388 )

♂ • spleen contains mainly myeloid-like cells and few lymphoid cells

decreased spleen B cell follicle number ( J:140388 )

♂ • spleen contains no follicles (or germinal centers)

abnormal adaptive immunity ( J:140388 )

♂ • mature T and B cell populations are absent

♂ • there are diminished CD3+CD4+ and CD3+CD8+ T cells and Igk+ light chain+ B cells in the spleen

abnormal innate immunity ( J:140388 )

♂ • cells functioning in innate immunity are severely decreased in number

abnormal response to transplant ( J:140388 )

♂ • 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)-Rag1^tm1Mom homozygotes

♂ • percentages of human CD45+ cells engrafted in bone marrow are higher than in NOD.129S7(B6)-Rag1^tm1Mom homozygotes

♂ • levels of human CD45+ cell and CD3+ T cell engraftment are higher in spleens than in NOD.129S7(B6)-Rag1^tm1Mom mice

♂ • 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.129S7(B6)-Rag1^tm1Mom mice

♂ • engraftment of human lymphohematopoietic cells in blood and thymus, and peripheral blood mononuclear cells (PMBC) engraftment are higher than in NOD.129S7(B6)-Rag1^tm1Mom mice

hematopoietic system

abnormal thymus morphology ( J:140388 )

♂ • no lymphoid cells are present

absent thymus cortex ( J:140388 )

♂ • no defined cortical region is observed

absent thymus medulla ( J:140388 )

♂ • no defined medullary region is observed

thymus cyst ( J:140388 )

♂ • thymus contains large cysts

absent NK T cells ( J:140388 )

♂ • natural killer cell (NK; DX5+LGL+ cells) are absent

decreased macrophage cell number ( J:140388 )

♂ • levels are Gr-1-ve Mac-1+ve macrophages are reduced

abnormal spleen morphology ( J:140388 )

♂ • spleen contains mainly myeloid-like cells and few lymphoid cells

decreased spleen B cell follicle number ( J:140388 )

♂ • spleen contains no follicles (or germinal centers)

endocrine/exocrine glands

abnormal thymus morphology ( J:140388 )

♂ • no lymphoid cells are present

absent thymus cortex ( J:140388 )

♂ • no defined cortical region is observed

absent thymus medulla ( J:140388 )

♂ • no defined medullary region is observed

thymus cyst ( J:140388 )

♂ • thymus contains large cysts

Genotype
MGI:3624447

ot13

• Allelic Composition: Il2rg^tm1Wjl/Y
• Genetic Background: involves: 129S4/SvJae * C57BL/6

immune system

cecum inflammation ( J:24117 )

♂ • cecum inflammation is visible in the lamina propia

abnormal lymphocyte morphology ( J:108209 )

♂

decreased double-negative T cell number ( J:24117 )

♂ • there is a reduced number of double-positive T cells in the thymus

decreased double-positive T cell number ( J:24117 )

♂ • there is a reduced number of double-negative T cells in the thymus

abnormal B cell number ( J:108209 )

♂ • after treatment with anti-Tpte, null mice show increased numbers of B cells in the spleen which contribute to the increased splenic cellularity

decreased immature B cell number ( J:24117 )

♂ • absolute numbers are reduced in the spleen

absent NK cells ( J:24117 )

♂ • NK1.1 positive cells are not detected

decreased CD8-positive, alpha-beta T cell number ( J:24117 )

♂ • 10- to 20- fold drop in percentage of CD8 positive T cells found in three week old spleen

♂ • percentages of CD8 positive T cells in spleen return to normal by 9 weeks of age

absent gamma-delta T cells ( J:24117 )

♂ • no gamma-delta T cells are found in the thymus or the skin

absent gamma-delta intraepithelial T cell ( J:24117 )

♂ • no gamma-delta IEL are found in the intestine

decreased single-positive T cell number ( J:24117 )

♂ • there is a reduced number of single-postive T cells in the thymus

abnormal CD4-positive, alpha beta T cell morphology ( J:108209 )

♂ • when Tpte-deficient CD4+ T cells are injected into irradiated knockouts, transferred cells exhibit less expansion compared to wild-type CD4+ T cells

abnormal immune system organ morphology ( J:108209 )

♂

small thymus cortex ( J:24117 )

♂ • cortex is reduced in size

thymus hyperplasia ( J:108209 )

♂ • after 1 week of daily injections of anti-Tpte, thymic cellularity is increased 5-10 fold in null mice; injections had no effect on cellularity in double knockouts

small thymus ( J:24117 )

♂ • thymus is very small

spleen hyperplasia ( J:24117 )

♂ • about a 2 fold increase in cellularity at nine weeks of age

spleen hypoplasia ( J:24117 )

♂ • about a 10 fold reduction in cellularity at three weeks of age

abnormal gut-associated lymphoid tissue morphology ( J:24117 )

♂ • gut associated lymphoid tissue was absent in the small and large intestine

abnormal mesenteric lymph node morphology ( J:24117 )

♂ • small, with few follicles and no germinal centers

small lymph nodes ( J:24117 )

♂ • peripheral lymph nodes are not identifiable

abnormal cell-mediated immunity ( J:108209 )

♂

decreased T cell proliferation ( J:24117 )

♂ • less potent proliferation of splenic T cells to mitogen stimulation

abnormal thymocyte activation ( J:24117 )

♂ • marked reduction in proliferation of thymocytes in response to PMA and IL-4 stimulation

abnormal basophil physiology ( J:24117 )

♂ • IL-4 production by basophil/mast cell incubation is increased 10- to 45- fold

abnormal mast cell physiology ( J:24117 )

♂ • IL-4 production by basophil/mast cell incubation is increased 10- to 45- fold

impaired natural killer cell mediated cytotoxicity ( J:24117 )

♂ • no NK cytolytic acitivity detected

abnormal immune serum protein physiology ( J:108209 )

♂

decreased immunoglobulin level ( J:24117 )

♂ • concentrations of all immunoglobulins but IgM are diminished in sera of 5-week old mice

decreased interferon-gamma secretion ( J:24117 )

♂ • IFN-gamma production by stimulated T cells is reduced by a 1000 fold

decreased interleukin-2 secretion ( J:24117 )

♂ • production of IL-2 by stimulated T cells is reduced by about 40%

decreased interleukin-4 secretion ( J:24117 )

♂ • production of IL-4 by stimulated T cells is reduced by about 40%

digestive/alimentary system

abnormal cecum morphology ( J:24117 )

♂ • crypt height is increased and inflammation is visible in the lamina propia

cecum inflammation ( J:24117 )

♂ • cecum inflammation is visible in the lamina propia

hematopoietic system

decreased T cell proliferation ( J:24117 )

♂ • less potent proliferation of splenic T cells to mitogen stimulation

small thymus cortex ( J:24117 )

♂ • cortex is reduced in size

thymus hyperplasia ( J:108209 )

♂ • after 1 week of daily injections of anti-Tpte, thymic cellularity is increased 5-10 fold in null mice; injections had no effect on cellularity in double knockouts

small thymus ( J:24117 )

♂ • thymus is very small

abnormal thymocyte activation ( J:24117 )

♂ • marked reduction in proliferation of thymocytes in response to PMA and IL-4 stimulation

spleen hyperplasia ( J:24117 )

♂ • about a 2 fold increase in cellularity at nine weeks of age

abnormal lymphocyte morphology ( J:108209 )

♂

decreased double-negative T cell number ( J:24117 )

♂ • there is a reduced number of double-positive T cells in the thymus

decreased double-positive T cell number ( J:24117 )

♂ • there is a reduced number of double-negative T cells in the thymus

abnormal B cell number ( J:108209 )

♂ • after treatment with anti-Tpte, null mice show increased numbers of B cells in the spleen which contribute to the increased splenic cellularity

decreased immature B cell number ( J:24117 )

♂ • absolute numbers are reduced in the spleen

absent NK cells ( J:24117 )

♂ • NK1.1 positive cells are not detected

decreased CD8-positive, alpha-beta T cell number ( J:24117 )

♂ • 10- to 20- fold drop in percentage of CD8 positive T cells found in three week old spleen

♂ • percentages of CD8 positive T cells in spleen return to normal by 9 weeks of age

absent gamma-delta T cells ( J:24117 )

♂ • no gamma-delta T cells are found in the thymus or the skin

absent gamma-delta intraepithelial T cell ( J:24117 )

♂ • no gamma-delta IEL are found in the intestine

decreased single-positive T cell number ( J:24117 )

♂ • there is a reduced number of single-postive T cells in the thymus

abnormal CD4-positive, alpha beta T cell morphology ( J:108209 )

♂ • when Tpte-deficient CD4+ T cells are injected into irradiated knockouts, transferred cells exhibit less expansion compared to wild-type CD4+ T cells

spleen hypoplasia ( J:24117 )

♂ • about a 10 fold reduction in cellularity at three weeks of age

abnormal basophil physiology ( J:24117 )

♂ • IL-4 production by basophil/mast cell incubation is increased 10- to 45- fold

abnormal mast cell physiology ( J:24117 )

♂ • IL-4 production by basophil/mast cell incubation is increased 10- to 45- fold

decreased immunoglobulin level ( J:24117 )

♂ • concentrations of all immunoglobulins but IgM are diminished in sera of 5-week old mice

impaired natural killer cell mediated cytotoxicity ( J:24117 )

♂ • no NK cytolytic acitivity detected

endocrine/exocrine glands

small thymus cortex ( J:24117 )

♂ • cortex is reduced in size

thymus hyperplasia ( J:108209 )

♂ • after 1 week of daily injections of anti-Tpte, thymic cellularity is increased 5-10 fold in null mice; injections had no effect on cellularity in double knockouts

small thymus ( J:24117 )

♂ • thymus is very small

abnormal thymocyte activation ( J:24117 )

♂ • marked reduction in proliferation of thymocytes in response to PMA and IL-4 stimulation

growth/size/body

spleen hyperplasia ( J:24117 )

♂ • about a 2 fold increase in cellularity at nine weeks of age

cellular

decreased T cell proliferation ( J:24117 )

♂ • less potent proliferation of splenic T cells to mitogen stimulation