Strain Name: |
NOD.Cg Prkdcscid-Tg(CSF2)2Ygy Tg(IL3)1Ygy Tg(KITLG)3Ygy/YgyJ |
|---|---|
Stock Number: |
004644 |
Availability: | Repository-Cryopreserved |
General Terms and Conditions |
| Former Name |
CB17.Cg-Prkdcscid Tg(IL3)1Ygy Tg(CSF2)2Ygy Tg(KITLG)3Ygy (Changed: 26-APR-06
) |
| Genes & Alleles | Prkdc; Prkdcscid; CMVP; CSF2; IL3; Tg(CSF2)2Ygy; Tg(IL3)1Ygy; Tg(KITLG)3Ygy; |
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 - Spontaneous Mutation Type JAX® GEMM® Strain - Transgenic Species laboratory mouse Background Strain NOD.CB17-Prkdcscid Donor Strain NIH/Swiss H2 Haplotype g7 Generation N7F?+N1F2+N1p (15-MAY-05) Appearance
albino, pink-eyed
Related Genotype: A/A Tyrc/TyrcStrain Description
Tg(IL3)1Ygy, Tg(CSF2)2Ygy, and Tg(KITLG)3Ygy encode porcine interleukin 3 (IL3), Porcine granulocyte macrophage-colony stimulating factor (CSF2, commonly designated GM-CSF) and soluble Porcine stem cell factor (KITLG, commonly designated sSCF) respectively. All three are individually driven by the human cytomegalovirus promoter. These three transgenes were co-injected and they co-segregate. RT-PCR detects transgenic expression of porcine IL-3, CSF2 and KITLG in bone marrow and spleen in NOD.Cg Prkdc scid-Tg(IL3)1Ygy Tg(CSF2)2Ygy Tg(KITLG)3Ygy mice. Porcine IL3, CSF2, and KITLG are present in the serum of these mice in levels that can be detected by ELISA. NOD.Cg Prkdc scid-Tg(IL3)1Ygy Tg(CSF2)2Ygy Tg(KITLG)3Ygy/YgyJ not only provide a system in which long-term porcine tissue and stem cell engraftment can be achieved (Abe et al, 2002) but also is a useful tool for evaluating donor-specific tolerance induction by mixed chimerism across xenogeneic barriers (Chen et al, 2000). White blood cells of NOD.Cg Prkdcscid-Tg(IL3)1Ygy Tg(CSF2)2Ygy Tg(KITLG)3Ygy mice are CD4-, CD8-, CD3-, IgM-, CD19- and Mac-1+ by flow cytometric (FMC) analysis. White blood cells of Tg(IL3)1Ygy Tg(CSF2)2Ygy Tg(KITLG)3Ygy mice sub-lethally irradiated, i.v. injected with miniature swine bone marrow cells and i.p. injected with peripheral blood mononuclear cells (PBMC) are swine myeloid CD-2+, class II+ and CD16+ 20 weeks post bone marrow transplant by FMC. Non-transgenic littermates had low levels of chimerism detected at 3 weeks and non-existent at 7 weeks post bone marrow transplant (BMT). At 20 weeks post BMT these transgenic mice revealed high levels of chimerism in the spleen and bone marrow cells, versus no chimerism in any tissue of non-transgenic littermates. Treated transgenic mice showed peak levels of porcine T cell chimerism at 7 weeks post transplantation. Neither B nor T cells were detectable at 20 weeks post transplantation (Chen et al, 2000).
Mammalian Phenotype Terms assigned by genotype |
Gene & Allele Details
| Allele Symbol | Prkdcscid | ||
|---|---|---|---|
| Allele Name | severe combined immunodeficiency | ||
| Common Name(s) | scid; | ||
| Strain of Origin | CB17 | ||
| 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] | ||
| Allele Symbol | Tg(CSF2)2Ygy | ||
| Allele Name | transgene insertion 2, Yong-guang Yang | ||
| Strain of Origin | NIH Swiss | ||
| Expressed Gene | CSF2, colony stimulating factor 2 (granulocyte-macrophage), pig, domestic | ||
| General Note | White blood cells from mice that carry this transgene in conjunction with Tg(IL3)1Ygy and Tg(KITLG)3Ygy and thatare also homozygous for Prkdcscid are CD4-, CD8-, CD3-, IgM-, CD19- and Mac-1+ by flow cytometric (FMC) analysis. | ||
| Molecular Note | The transgene construct contains the porcine colony stimulating factor 2 (granulocyte-macrophage) sequence (CSF2) controlled by the human cytomegalovirus promoter. The Tg(IL3)1Ygy, Tg(CSF2)2Ygy and Tg(KITLG)3Ygy transgenic constructs were co-injected into NIH/Swiss oocytes in David Bodine's lab at NCI. [MGI Ref ID J:94302] | ||
| Allele Symbol | Tg(IL3)1Ygy | ||
| Allele Name | transgene insertion 1, Yong-guang Yang | ||
| Strain of Origin | NIH Swiss | ||
| Expressed Gene | IL3, interleukin 3 (colony-stimulating factor, multiple), pig, domestic | ||
| General Note | White blood cells from mice that carry this transgene in conjunction with Tg(CSF2)2Ygy and Tg(KITLG)3Ygy and that are also homozygous for Prkdcscid are CD4-, CD8-, CD3-, IgM-, CD19- and Mac-1+ by flow cytometric (FMC) analysis. | ||
| Molecular Note | The transgene construct contains the porcine interleukin 3 sequence controlled by the human cytomegalovirus promoter. The Tg(IL3)1Ygy, Tg(CSF2)2Ygy and Tg(KITLG)3Ygy transgenic constructs were co-injected into NIH/Swiss oocytes in David Bodine's lab at NCI. [MGI Ref ID J:94302] | ||
| Allele Symbol | Tg(KITLG)3Ygy | ||
| Allele Name | transgene insertion 3, Yong-guang Yang | ||
| Strain of Origin | NIH Swiss | ||
| Promoter | CMVP, cytomegalovirus, human | ||
| General Note | White blood cells from mice that carry this transgene in conjunction with Tg(IL3)1Ygy and Tg(CSF2)2Ygy and thatare also homozygous for Prkdcscid are CD4-, CD8-, CD3-, IgM-, CD19- and Mac-1+ by flow cytometric (FMC) analysis. | ||
| Molecular Note | The transgene construct contains porcine kit ligand (KITL) controlled by the human cytomegalovirus promoter. The Tg(IL3)1Ygy, Tg(CSF2)2Ygy and Tg(KITLG)3Ygy transgenic constructs were co-injected into NIH/Swiss oocytes in David Bodine's lab at NCI. [MGI Ref ID J:94302] | ||
Control Information
| Control | ||
|---|---|---|
| Noncarrier | ||
| 001303 NOD.CB17-Prkdcscid/J | ||
| Considerations for Choosing Controls | ||
Genotyping Protocols
Prkdcscid
Colony Maintenance
| Diet Information | LabDiet® 5K52/5K67 |
|---|
Related Strains
Strains carrying Prkdcscid allele
View Strains carrying Prkdcscid (25 strains)
Additional Web Information
Congenic Nomenclature
Research Applications
This mouse can be used to support research in many areas including:
Prkdcscid relatedDiabetes and Obesity Research
Type 1 Diabetes (IDDM)
Immunology and Inflammation Research
T Cell Receptor Signaling Defects (B and T cell deficiency) (xenograft/transplant host)
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)
Additional ReferencesChen AM; Zhou Y; Swenson K; Sachs DH; Sykes M; Yang YG. 2000. Porcine stem cell engraftment and seeding of murine thymus with class II+ cells in mice expressing porcine cytokines: toward tolerance induction across discordant xenogeneic barriers. Transplantation 69(12):2484-90. [PubMed: 10910267] [MGI Ref ID J:93486]
Price and Supply Information
| Strain Name: | NOD.Cg Prkdcscid-Tg(CSF2)2Ygy Tg(IL3)1Ygy Tg(KITLG)3Ygy/YgyJ |
| Stock Number: | 004644 |
Price Details
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Supply Details
| Standard Supply | Repository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information. |
|---|---|
| Supply Notes |
Cryopreserved Embryos This strain is also available as cryopreserved embryos from our Repository. Orders for cryopreserved embryos are supplied subject to a signed agreement that must be returned to the Customer Service Department after order placement. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos from our repository, please visit our Cryopreserved Embryos web page. 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. |
| Licensing | See General Terms and Conditions below for Licensing and Use Restrictions |
| Control Information | View Control Information in Strain Details. |
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