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Strain Name:

NOD.Cg-Rag1tm1Mom Prf1tm1Sdz/SzJ

Stock Number:

004848

Availability:

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Former Name      NOD.Cg-Rag1tm1Mom Prf1tm1Sdz/Sz    (Changed: 15-DEC-04 )
Genes & Alleles   Prf1;   Prf1tm1Sdz;   Rag1;   Rag1tm1Mom;

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 - Targeted Mutation
Mating SystemHomozygote x Homozygote         (Female x Male)
Specieslaboratory mouse
Donating Investigator Leonard Shultz,   The Jackson Laboratory
H2 Haplotypeg7
GenerationN10F7 (05-DEC-07)

Strain Description
Mice that are homozygous for both targeted mutations are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities when housed under specific pathogen free conditions. These double homozygote mutant mice have no mature T or B lymphocytes, no detectable NK cell cytotoxic activity, and lack serum immunoglobulin. The number of nucleated spleen cells is significantly reduced in double mutant mice, when compared to the single homozygote, NOD.129S7(B6)-Rag1tm1Mom/J (Stock No. 003729). Although an increased number of DX5+CD122+ NK cells are found in the spleens of double mutants, these NK cells have impaired cytotoxic activity. The disruption of Prf1 ablates NK cell cytotoxic activity resulting in increased engraftment levels over that observed with Stock No. 003729. All mutant mice develop thymic lymphomas. This double mutant mouse strain may be useful in studies involving engraftment of human hematolymphoid cells.

Strain Development
Please see strain entries Stock No. 002407 and Stock No. 003729 for details regarding the construction of each mutant allele. This strain was produced by crossing female NOD.129S7(B6)-Rag1tm1Mom/J (Stock No. 003729) to male C57BL/6-Prf1tm1Sdz (Stock No. 002407). Resulting female progeny heterozygous for both mutant alleles were crossed to male NOD.129S7(B6)-Rag1tm1Mom/J (Stock No. 003729). Mice homozygous for the Rag1 mutant allele and heterozygous for the Prf1 allele were crossed to NOD.129S7(B6)-Rag1tm1Mom/J (Stock No. 003729) mice for 10 generations, and then intercrossed to produce double homozygotes.

Mammalian Phenotype Terms assigned by genotype

Rag1tm1Mom/Rag1tm1Mom Prf1tm1Sdz/Prf1tm1Sdz

        NOD.Cg-Rag1tm1Mom Prf1tm1Sdz/Sz
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:109843)
    • mean lifespan is 262+/-21 days
  • immune system phenotype
  • abnormal level of surface class II molecules (MGI Ref ID J:109843)
    • four to five-fold decrease in cells expressing I-Ag7 as compared to NOD control
  • abnormal lymph node cortex (MGI Ref ID J:109843)
    • lymph nodes lack follicles
  • abnormal response to transplant (MGI Ref ID J:109843)
    • following IP injection of human PMBC, peripheral blood has a fivefold increase in the percentage of human lymphoid cells as compared to NOD-Ragnull controls
    • peripheral blood has a 13-fold increase in engraftment of human CD4+ T cells
    • spleens have a 9-fold increase in engraftment of human CD45+ cells and a 20-fold increase in human CD4+ cells as compared to control, although there is no significant difference in engraftment of human CD8+ cells
    • 6-8 weeks after IP injection, bone marrow exhibits a 12-fold increase in engraftment of human cord blood cells
  • abnormal spleen white pulp morphology (MGI Ref ID J:109843)
    • spleen lacks lymphoid follicles
  • abnormal thymus cortex morphology (MGI Ref ID J:109843)
    • thymus lacks a clearly defined cortex
  • decreased CD4-positive T cell number (MGI Ref ID J:109843)
    • spleens of 8-11 week old mice are deficient in CD3+ CD4+ T cells
  • decreased CD8-positive T cell number (MGI Ref ID J:109843)
    • spleens of 8-11 week old mice are deficient in CD3+ CD8+ T cells
  • decreased immunoglobulin level (MGI Ref ID J:109843)
    • no serum immunoglobulin is detected in 176-362 day old mice
  • decreased mature B cell number (MGI Ref ID J:109843)
    • spleens of 8-11 week old mice are deficient in Igkappa+ B220+ T cells
  • impaired NK cell cytolysis (MGI Ref ID J:109843)
    • poly I:C stimulated spleen cells exhibit extremely low levels of NK cell cyotoxic activity
  • increased NK cell number (MGI Ref ID J:109843)
    • numbers of NK cells are increased in comparison to NOD controls
  • increased granulocyte number (MGI Ref ID J:109843)
    • numbers of granulocytes are increased in comparison to NOD controls, however, in comparison to NOD-Ragnull numbers are decreased
  • increased immature B cell number (MGI Ref ID J:109843)
    • IgLkappa- B220+ immature B cells are increased six-fold as compared to NOD controls
  • increased macrophage cell number (MGI Ref ID J:109843)
    • numbers of macrophages are increased in comparison to NOD controls
  • spleen hypoplasia (MGI Ref ID J:109843)
    • nucleated spleen cells are reduced 14 fold in 8-11 week old mice as compared to NOD control
  • thymus hypoplasia (MGI Ref ID J:109843)
    • lymphoid cells are severely reduced in thymuses
  • tumorigenesis
  • thymic lymphoma (MGI Ref ID J:109843)
  • hematopoietic system phenotype
  • abnormal spleen white pulp morphology (MGI Ref ID J:109843)
    • spleen lacks lymphoid follicles
  • abnormal thymus cortex morphology (MGI Ref ID J:109843)
    • thymus lacks a clearly defined cortex
  • decreased CD4-positive T cell number (MGI Ref ID J:109843)
    • spleens of 8-11 week old mice are deficient in CD3+ CD4+ T cells
  • decreased CD8-positive T cell number (MGI Ref ID J:109843)
    • spleens of 8-11 week old mice are deficient in CD3+ CD8+ T cells
  • decreased mature B cell number (MGI Ref ID J:109843)
    • spleens of 8-11 week old mice are deficient in Igkappa+ B220+ T cells
  • increased NK cell number (MGI Ref ID J:109843)
    • numbers of NK cells are increased in comparison to NOD controls
  • increased granulocyte number (MGI Ref ID J:109843)
    • numbers of granulocytes are increased in comparison to NOD controls, however, in comparison to NOD-Ragnull numbers are decreased
  • increased immature B cell number (MGI Ref ID J:109843)
    • IgLkappa- B220+ immature B cells are increased six-fold as compared to NOD controls
  • increased macrophage cell number (MGI Ref ID J:109843)
    • numbers of macrophages are increased in comparison to NOD controls
  • spleen hypoplasia (MGI Ref ID J:109843)
    • nucleated spleen cells are reduced 14 fold in 8-11 week old mice as compared to NOD control
  • thymus hypoplasia (MGI Ref ID J:109843)
    • lymphoid cells are severely reduced in thymuses

Gene & Allele Details

Allele Symbol Prf1tm1Sdz
Allele Name targeted mutation 1, Sandoz Pharmaceutical
Common Name(s) P0; Pfptm1Sdz; Prf1-; perf-; perforin 0; pfp-; pko;
Mutation Made By Birgit Lederman,   University of Zurich
Strain of OriginC57BL/6
ES Cell Line NameBL/6-III
ES Cell Line StrainC57BL/6
Gene Symbol and Name Prf1, perforin 1 (pore forming protein)
Chromosome 10
Gene Common Name(s) Cyta; FLH2; HPLH2; MGC108712; MGC65093; P1; PFN1; PFP; Pfp; Prf-1; RATCYTA; perforin; perforin 1; pore forming protein;
Molecular Note A neomycin selection cassette was inserted into exon 3. RT-PCR analysis on RNA derived from homozygous mice demonstrated that an abnormal transcript was produced from this allele. However, immunocytochemistry experiments on activated spleen cells derived from homozygous mice confirmed that no detectable protein was made from this allele. [MGI Ref ID J:17986] [MGI Ref ID J:96542]
 
Allele Symbol Rag1tm1Mom
Allele Name targeted mutation 1, Peter Mombaerts
Common Name(s) RAG-1-; RAG1null; Rag-; Rag-1KO; Rag1-;
Mutation Made By Peter Mombaerts,   Max Planck Institute of Biophysics
Strain of Origin129S7/SvEvBrd-Hprt1<+>
ES Cell Line NameAB1
ES Cell Line Strain129S7/SvEvBrd-Hprt1<+>
Gene Symbol and Name Rag1, recombination activating gene 1
Chromosome 2
Gene Common Name(s) MGC43321; RNF74; Rag-1;
Molecular Note A 1356 bp genomic fragment of the Rag1 gene, encoding the nuclear localization signal and the zinc-finger motif, was replaced by a neomycin cassette. A mutant transcript expressed from this allele was detected by Northern blot in bone marrow derived cell lines from homozygous mice. [MGI Ref ID J:1934] [MGI Ref ID J:96036]

Control Information

  Control
   001976 NOD/ShiLtJ (approximate)
 
  Considerations for Choosing Controls

Genotyping Protocols

Prf1tm1Sdz
Rag1tm1Mom

Colony Maintenance

Breeding & HusbandryThis strain is maintained as a homozygote for both mutant alleles, under specific pathogen free conditions.
Diet Information LabDiet® 5K52/5K67

Related Strains

Strains carrying   Prf1tm1Sdz allele
002407   C57BL/6-Prf1tm1Sdz/J
003505   NOD.B6-Prf1tm1Sdz/J
View Strains carrying   Prf1tm1Sdz     (2 strains)

View Strains carrying   Rag1tm1Mom     (16 strains)

Strains carrying other alleles of Rag1
002194   B6;CBA-Tg(CD3E)26Cpt/J
002506   STOCK Tg(CD3E)26Cpt-Rag1tm1Mom/J
View Strains carrying other alleles of Rag1     (2 strains)

Additional Web Information

Congenic Nomenclature
JAX® NOTES, Spring 2006; 501. Choosing an Immunodeficient Mouse Model.

Animal Health Reports

Room Number           AX12

Research Applications

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

Cancer Research
Increased Tumor Incidence (Lymphomas: thymic)
Toxicology (B and T cell deficiency) (xenograft/transplant host)

Developmental Biology Research
Lymphoid Tissue Defects (hematopoietic defects)

Hematological Research
Immunological Defects (B and T cell deficiency)

Immunology and Inflammation Research
Autoimmunity (B and T cell deficiency)
Autoimmunity (B cell deficiency)
Immunodeficiency (B and T cell deficiency)
Immunodeficiency (B cell deficiency)
Immunodeficiency (B, T, and NK cell deficiency)
Immunodeficiency (NK Cell Deficiency)
Immunodeficiency (multiple immune defects)
Inflammation (B and T cell deficiency)
Lymphoid Tissue Defects (B and T cell deficiency)
Lymphoid Tissue Defects (hematopoietic development)

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

Research Tools
Cancer Research (B and T cell deficiency) (xenograft/transplant host)
Cancer Research (B cell deficiency)
Cancer Research (B, T, and NK cell deficiency) (xenograft/transplant host)
Cancer Research (T cell deficiency)
Cancer Research (genes regulating lymphoma development)
Cancer Research (production of B and T cells, antibodies, and hybridomas)
Cancer Research (xenograft/transplant host)
Immunology and Inflammation Research (B and T cell deficiency)
Immunology and Inflammation Research (B cell deficiency)
Immunology and Inflammation Research (B, T, and NK cell deficiency)
Immunology and Inflammation Research (NK Cell Deficiency)
Immunology and Inflammation Research (T cell deficiency)
Immunology and Inflammation Research (T cell deficiency) (xenograft/transplant host)
Immunology and Inflammation Research (production of B cells, antibodies T cell lines, and hybridomas)
Immunology and Inflammation Research (production of T cell lines and hybridomas)
Toxicology Research (B and T cell deficiency) (xenograft transplant host)
Toxicology Research (xenograft/transplant host)

Prf1tm1Sdz related

Apoptosis Research
Extracellular Modulators

Immunology and Inflammation Research
Immunodeficiency Associated with Other Defects

Rag1tm1Mom related

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

Hematological Research
Immunological Defects (B and T cell deficiency)

Immunology and Inflammation Research
Immunodeficiency (B and T cell deficiency)
Inflammation (B and T cell deficiency)
T Cell Receptor Signaling Defects (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)

References

Selected Reference(s)

Shultz LD; Banuelos S; Lyons B; Samuels R; Burzenski L; Gott B; Lang P; Leif J; Appel M; Rossini A; Greiner DL. 2003. NOD/LtSz-Rag1nullPfpnull mice: a new model system with increased levels of human peripheral leukocyte and hematopoietic stem-cell engraftment. Transplantation 76(7):1036-42. [PubMed: 14557749]  [MGI Ref ID J:109843]

Additional References

Price and Supply Information

Strain Name: NOD.Cg-Rag1tm1Mom Prf1tm1Sdz/SzJ
Stock Number: 004848

Price Details

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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 Usually shipped between four and eight 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.
LicensingSee General Terms and Conditions below for Licensing and Use Restrictions  
Control InformationView Control Information in Strain Details.

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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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