Strain Name: |
NOD.Cg-Prkdcscid Tg(TcrLCMV)327Sdz/Dvs |
|---|---|
Stock Number: |
004257 |
Availability: | Research Strain |
General Terms and Conditions |
| Genes & Alleles | Prkdc; Prkdcscid; Tcra; Tcrb; Tg(TcrLCMV)327Sdz; |
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 - Transgenic Species laboratory mouse H2 Haplotype g7 Generation N2F19 (06-DEC-05) Appearance
albino, pink eyed
Related Genotype: A/A Tyrc/Tyrc
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(TcrLCMV)327Sdz | ||
| Allele Name | transgene insertion 327, Birgit Ledermann | ||
| Common Name(s) | P14; P14 TCR; P14 TCR-alphabeta (Valpha2Vbeta8.1); P14 TCRValpha2Vbeta8; P14-TCR; TCR LCMV; TCR P14; TCR-Tg; TgTcr; | ||
| Mutation Made By | Rolf Zinkernagel, University of Zurich | ||
| Strain of Origin | (C57BL/6 x DBA/2)F2 | ||
| Expressed Gene | Tcrb, T-cell receptor beta chain, mouse, laboratory | ||
| Expressed Gene | Tcra, T-cell receptor alpha chain, mouse, laboratory | ||
| General Note | Transgenic mice carry a T-cell receptor (Tcra-V2, Tcra-J TA31 / Tcrb-V8.1, Tcrb-D, Tcrb-J 2.4) specific for LCMV (lymphocytic choriomeningitis virus), H2-Db. | ||
| Molecular Note | The transgene carries both Tcra and Tcrb. Ten to twenty copies of the Tcr transgene were reported to have integrated on the same chromosome. At least two transgenic lines are known to exist. Line 327 is the representative line. The second line is 318. [MGI Ref ID J:77696] | ||
Genotyping Protocols
Prkdcscid
Tg(TcrLCMV)327Sdz
Related Strains
Strains carrying Prkdcscid allele
View Strains carrying Prkdcscid (25 strains)
004694 B6;D2-Tg(TcrLCMV)327Sdz/JDvsJ 004696 NOD.Cg-Tg(TcrLCMV)327Sdz/DvsJ
Additional Web Information
Congenic Nomenclature
Research Applications
This mouse can be used to support research in many areas including:
Tcra relatedResearch Tools
Immunology and Inflammation Research (T Cell Receptor Transgenics)
Tcrb relatedHematological Research
Immunological Defects
Immunology and Inflammation Research
CD Antigens, Antigen Receptors, and Histocompatibility Markers
Immunodeficiency
Inflammation
T Cell Receptor Signaling Defects
Research Tools
Cancer Research (specific T cell deficiency)
Prkdcscid relatedHematological Research
Immunological Defects
Immunology and Inflammation Research
CD Antigens, Antigen Receptors, and Histocompatibility Markers
Immunodeficiency
Inflammation
T Cell Receptor Signaling Defects
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 ReferencesSerreze DV; Johnson EA; Chapman HD; Graser RT; Marron MP; DiLorenzo TP; Silveira P; Yoshimura Y; Nathenson SG; Joyce S. 2001. Autoreactive diabetogenic T-cells in NOD mice can efficiently expand from a greatly reduced precursor pool. Diabetes 50(9):1992-2000. [PubMed: 11522664] [MGI Ref ID J:71050]
Price and Supply Information
| Strain Name: | NOD.Cg-Prkdcscid Tg(TcrLCMV)327Sdz/Dvs |
| Stock Number: | 004257 |
Price Details
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Supply Details
| Standard Supply | Research Strain. Availability determined by The Jackson Laboratory scientist holding the strain. |
|---|---|
| Licensing | See General Terms and Conditions below |
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