Strain Name: |
CB17;HPG-Prkdcscid Gnrh1hpg/Bm |
|---|---|
Stock Number: |
002038 |
Availability: | Research Strain |
General Terms and Conditions |
| Former Name |
CB17;HPG-Prkdcscid Gnrh2hpg/Bm (Changed: 27-FEB-06
) |
|
CB17;HPG-Prkdcscid Gnrhhpg/Bm (Changed: 08-JUL-05
) | |
| Genes & Alleles | Gnrh1; Gnrh1hpg; Prkdc; Prkdcscid; |
Product Information
Strain Details
Type JAX® GEMM® Strain - Mutant Strain Additional information on JAX® GEMM® Strains. Species laboratory mouse Generation N2F30 (16-NOV-05) Strain Description
Mice homozygous for the severe combined immune deficiency spontaneous mutation (Prkdcscid, commonly referred to as scid) are characterized by an absence of functional T cells and B cells, lymphopenia, hypogammaglobulinemia, and a normal hematopoietic microenvironment. Normal antigen-presenting cell, myeloid and NK cell functions are strain dependent. scid mice carry a DNA repair defect and a defect in the rearrangement of genes that code for antigen-specific receptors on lymphocytes. Most homozygotes have no detectable IgM, IgG1, IgG2a, IgG2b, IgG3, or IgA. Thymus, lymph nodes, and splenic follicles are virtually devoid of lymphocytes. scid mice accept allogeneic and xenogeneic grafts making them an ideal model for cell transfer experiments. Some scid mice will spontaneously develop partial immune reactivity. scid mice that have serum Ig levels greater than 1 ug/ml are considered "leaky." scid leakiness is highly strain dependent, increases with age, and is higher in mice housed under non SPF conditions. In general, scid leakiness is high on the C57BL/6J and BALB/cBy genetic backgrounds, low on the C3H/HeJ background, and even lower on the NOD/LtSz background. Hypogonadal mice (Ghrhhpg) mice are deficient in gonadotropins and gonodal steroids leading to an overall underdevelopment of the reproductive tract. Male mice have a small penis and scrotum and a short ano-genital distance. All male reproductive organs are present but immature. The testes are small and undescended. Spermatogenesis is arrested, usually by the diplotene stage. The vagina of female mice does not open fully, the uterus is thread-like, and the ovaries very small. Both sexes are sterile although fertility can be restored with hormone replacement. Ghrhhpg Prkdcscid double mutant mice are useful for determining endocrine dependence of grafted cells/tissues.
Gene & Allele Details
| Allele Symbol | Gnrh1hpg | ||
|---|---|---|---|
| Allele Name | hypogonadal | ||
| Common Name(s) | gnrhhpg; hpg; | ||
| Strain of Origin | (C3H/HeH x 101/H)F1-Rb(1.3)1Bnr | ||
| Gene Symbol and Name | Gnrh1, gonadotropin releasing hormone 1 | ||
| Chromosome | 14 | ||
| Gene Common Name(s) | GNRH; GRH; LHRH; LNRH; hpg; hypogonadal; | ||
| Molecular Note | The mutation in the hypogonadal mouse is an approximately 33.5 kb deletion that removes two exons that encode most of the protein. [MGI Ref ID J:14505] [MGI Ref ID J:8515] [MGI Ref ID J:94662] | ||
| 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] | ||
Control Information
| Allele | Control | |
|---|---|---|
| Gnrh1hpg | Untyped from the colony | |
| Considerations for Choosing Controls | ||
Genotyping Protocols
Gnrh1hpg
Prkdcscid
Colony Maintenance
| Breeding & Husbandry | Comments: homozygotes are sterile due to underdeveloped reproductive tracts. |
|---|
Related Strains
Strains carrying Gnrh1hpg allele
000804 HPG/BmJ View Strains carrying Gnrh1hpg (1 strain)
Research Applications
This mouse can be used to support research in many areas including:
Gnrh1hpg relatedResearch Tools
Immunology and Inflammation Research (B and T cell deficiency)
Prkdcscid relatedEndocrine Deficiency Research
Hypothalamus/Pituitary Defects
Reproductive Biology Research
Developmental Defects Affecting Gonads
Endocrine Deficiencies Affecting Gonads
Fertility Defects (males only)
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
Additional ReferencesPrice and Supply Information
| Strain Name: | CB17;HPG-Prkdcscid Gnrh1hpg/Bm |
| Stock Number: | 002038 |
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 |
| Control Information | View Control Information in Strain Details. |
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