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Strain Name:

NOD.Cg-Prkdcscid B2mtm1Unc/J

Stock Number:

002570

Availability:

Level 4

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General Terms and Conditions

Genes & Alleles   B2m;   B2mtm1Unc;   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 - Targeted Mutation
Mating SystemHomozygote x Homozygote         (Female x Male)
Specieslaboratory mouse
Background Strain NOD/LtSz-Prkdcscid
Donor Strain B6;129P-B2mtm1Unc (129P2 derived E14TG2a ES cell line)
Donating Investigator Leonard Shultz,   The Jackson Laboratory
H2 Haplotypeg7
GenerationN10F?+30 (20-DEC-06)

Appearance
albino
Related Genotype: Tyrc/Tyrc

Strain Description
Mice homozygous for both the B2mtm1Unc and Prkdcscid (commonly referred to as scid) mutations on the NOD/ShiLtSz background are class I deficient, B and T cell deficient, C-5 deficient (Hc0), and have low NK cells. This strain is an ideal model for xenograft transplantation studies and is an excellent source for insulitis-free, MHC class I-negative islets for transplantation studies.

Strain Development
The B2mtm1Unc targeted mutant strain was developed in the laboratory of Dr. Beverly Koller and Dr. Oliver Smithies at the University of North Carolina at Chapel Hill. It was generated by a targeted disruption of the B2m gene. The 129-derived E14TG2a ES cell line was used. This strain carrying both B2m and scid mutations was generated in the laboratory of Dr. Leonard Shultz at the Jackson Laboratory by backcrossing the B2mtm1Unc mutation 10 generations to the NOD/LtSz-Prkdcscid strain.

Mammalian Phenotype Terms assigned by genotype

B2mtm1Unc/B2mtm1Unc Prkdcscid/Prkdcscid

        NOD.Cg-B2mtm1Unc Prkdcscid
  • immune system phenotype
  • abnormal NK cell physiology (MGI Ref ID J:102141)
    • lacks detectable NK cell activity after pretreatment with NK cell inducer
  • abnormal response to transplant (MGI Ref ID J:102141)
    • mouse supports high levels of human CD4+ T cell engraftment in spleen and peripheral blood following human PBMC injection
    • minimal engraftment of human CD19+ B cells (less than 2%) following human PBMC injection
    • rapid clearance of human IgG
  • absent lymphocyte (MGI Ref ID J:102141)
    • lacks lymphoid cells in the spleen, lymph nodes and thymus
  • arrested B cell differentiation (MGI Ref ID J:102141)
    • absence of mature B cells in spleen, very little serum Ig
  • arrested T cell differentiation (MGI Ref ID J:102141)
    • few CD4+ and CD8+ cells in spleen and no mature T cells
  • decreased level of surface class I molecules (MGI Ref ID J:102141)
  • increased macrophage cell number (MGI Ref ID J:102141)
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:102141)
    • mean life span is 191+11 days
  • tumorigenesis
  • thymic lymphoma (MGI Ref ID J:102141)
    • associated with shortened lifespan
  • homeostasis/metabolism phenotype
  • increased liver iron level (MGI Ref ID J:102141)
    • intracytoplasmic iron deposition in liver
  • hematopoietic system phenotype
  • absent lymphocyte (MGI Ref ID J:102141)
    • lacks lymphoid cells in the spleen, lymph nodes and thymus
  • arrested B cell differentiation (MGI Ref ID J:102141)
    • absence of mature B cells in spleen, very little serum Ig
  • arrested T cell differentiation (MGI Ref ID J:102141)
    • few CD4+ and CD8+ cells in spleen and no mature T cells
  • increased macrophage cell number (MGI Ref ID J:102141)
  • liver/biliary system phenotype
  • increased liver iron level (MGI Ref ID J:102141)
    • intracytoplasmic iron deposition in liver

Gene & Allele Details

Allele Symbol B2mtm1Unc
Allele Name targeted mutation 1, University of North Carolina
Common Name(s) I0; b2mnull; beta2-m-KO; beta2M-; beta2MKO; beta2m0; beta2mnull; beta2mo; beta2mtm1Unc;
Mutation Made By Oliver Smithies,   University of North Carolina
Strain of Origin129P2/OlaHsd
ES Cell Line NameE14TG2a
ES Cell Line Strain129P2/OlaHsd
Gene Symbol and Name B2m, beta-2 microglobulin
Chromosome 2
Gene Common Name(s) Ly-m11; beta 2 microglobulin; beta2-m; lymphocyte antigen m11;
Molecular Note Insertion of a neomycin-resistance gene into the second exon. [MGI Ref ID J:65612]
 
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
   001976 NOD/ShiLtJ
   Additional control strains are available depending on the researchers needs. Please refer to JAX Notes No. 477 for a complete list of control strains available for NOD/LtJ mice in diabetes research. JAX Notes .
 
  Considerations for Choosing Controls

Genotyping Protocols

B2mtm1Unc
Prkdcscid

Colony Maintenance

Breeding & HusbandryThis B2mtm1Unc strain is maintained by mating homozygous siblings. Only homozygous mice may be ordered. Reproduction is good. Expected coat color from breeding:Albino
Diet Information LabDiet® 5K52/5K67

Related Strains

View Strains carrying   B2mtm1Unc     (19 strains)

View Strains carrying   Prkdcscid     (25 strains)

Additional Web Information

Congenic Nomenclature
Genetic Quality Control Annual Report
JAX® NOTES, Fall 1999; 479. New Scid Model.
JAX® NOTES, Spring 2003; 489. Role of NK and NKT Cells in Immunity and Disease.
JAX® NOTES, Spring 2006; 501. Choosing an Immunodeficient Mouse Model.

Animal Health Reports

Room Number           AX30

Research Applications

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

Diabetes and Obesity Research
Type 1 Diabetes (IDDM) Analysis Strains (NOD Congenics with Mutations Affecting Immunocompetence)

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

B2mtm1Unc related

Hematological Research
Anemia, Iron Deficiency and Transport Defects (hemochromatosis)

Immunology and Inflammation Research
Immunodeficiency (MHC class I deficiency)

Internal/Organ Research
Liver Defects (hemochromatosis)

Metabolism Research
Hemochromatosis (iron metabolism defects)

Research Tools
Immunology and Inflammation Research (MHC class I deficiency)

Prkdcscid related

Immunology and Inflammation Research
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)

Christianson SW; Greiner DL; Hesselton RA; Leif JH; Wagar EJ; Schweitzer IB; Rajan TV; Gott B; Roopenian DC; Shultz LD. 1997. Enhanced human CD4+ T cell engraftment in beta2-microglobulin-deficient NOD-scid mice. J Immunol 158(8):3578-86. [PubMed: 9103418]  [MGI Ref ID J:102141]

Additional References

Price and Supply Information

Strain Name: NOD.Cg-Prkdcscid B2mtm1Unc/J
Stock Number: 002570

Price Details

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Supply Details

Standard SupplyLevel 4. Up to 10 mice. Larger quantities or custom orders arranged upon request. Expected delivery up to one to three months.
Supply Notes 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.
LicensingSee General Terms and Conditions below  
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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