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Strain Name:

NOD.Cg-Prkdcscid Gusbmps/SndsJ

Stock Number:

005053

Availability:

Repository- Live

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General Terms and Conditions

Former Name      NOD.Cg-Prkdcscid-Gusbmps/SndsJ    (Changed: 20-DEC-04 )
      NOD.Cg Prkdcscid-Gusmps/SandsJ    (Changed: 15-DEC-04 )
      NOD.Cg-Prkdcscid-Gusmps/SndsJ    (Changed: 15-DEC-04 )
      NOD.Cg-Prkdcscid-Gusmps/WumJ    (Changed: 15-DEC-04 )
Genes & Alleles   Gusb;   Gusbmps;   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 - Spontaneous Mutation
Mating SystemHeterozygote x Heterozygote         (Female x Male)
Specieslaboratory mouse
Background Strain NOD.CB17-Prkdcscid/J
Donor Strain B6.C-H2bm1/ByBir-Gusmps/J 000256
GenerationN13F?+F7 (06-DEC-07)

Appearance
albino
Related Genotype: A/A Tyrc/Tyrc

Important Note
This strain is homozygous for Prkdcscid.

Strain Description
Mice homozygous for the mps allele exhibit skeletal dysplasia as well as cognitive, hearing and visual deficits. Lifespan of the homozygotes is approximately six months. Homozygotes lack the lysomal enzyme, beta-glucoronidase, and, as a result, glycosaminoglycans accumulate in tissues throughout the body. This strain is a model for the human lysomal storage disease, mucopolysaccharidosis type VII. On the NOD-Prkdcscid background, this strain can be used for xenotransplantation experiments and cell trafficking studies (Hofling et al., 2003).

Strain Development
The donating investigator, Dr. Mark Sands (Snds) at Washington University, St. Louis, generated this strain by backcrossing the Gusmps allele from B6.C-H2bm1/ByBir-Gusmps/J (Stock#000256) to NOD.CB17-Prkdcscid/J (Stock# 001303) for greater than ten generations. Both strains were obtained from The Jackson Laboratory. The mps mutation arose spontaneously on B6.C-H2bm1/By in 1976 (Birkenmeier et al., 1989). Dr. Sands donated this strain to The Jackson Laboratory in 2004.

Gene & Allele Details

Allele Symbol Gusbmps
Allele Name beta glucuronidase, mucopolysaccharidosis VII
Common Name(s) Gus-b; asd; gusmps;
Mutation Made By Brian Soper,   The Jackson Laboratory
Strain of OriginC57BL/6By
Gene Symbol and Name Gusb, glucuronidase, beta
Chromosome 5
Gene Common Name(s) AI747421; Ac2-223; FLJ39445; Gur; Gus; Gus-r; Gus-s; Gus-t; Gus-u; Gut; MPS7; adipose storage deficiency; asd; beta-glucuronidase regulator; beta-glucuronidase structural; beta-glucuronidase systemic regulator; beta-glucuronidase temporal; expressed sequence AI747421; g;
Molecular Note A 1-bp deletion creates a frameshift mutation within exon 10, which introduces a premature stop codon at codon 497. [MGI Ref ID J:13207]
 
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
   Wild-type from the colony
   002312 NOD.CB17-Prkdcscid
 
  Considerations for Choosing Controls

Genotyping Protocols

Prkdcscid

Colony Maintenance

Diet Information LabDiet® 5K20

Related Strains

View Strains carrying   Gusbmps     (7 strains)

View Strains carrying   Prkdcscid     (25 strains)

Strains carrying other alleles of Gusb
005643   B6.129X-Gusbtm1Sly/J
005644   B6.129X-Gusbtm3Sly/J
006557   B6.C3-Gusbmps-2J/BrkJ
006558   B6.Cg-H2bm1 Tg(GUSB)4Sly/SndsJ
003525   C3H/HeOuJ-Gusbmps-2J/BrkJ
005322   C57BL/6J-Gusbmps-3J/J
001880   STOCK Gusbmps Tg(GUSB)4Sly/BirJ
View Strains carrying other alleles of Gusb     (7 strains)

Additional Web Information

Congenic Nomenclature

Animal Health Reports

Room Number           AX12

Research Applications

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

Gusbmps related

Developmental Biology Research
Growth Defects Growth Defects (homozygous)
Skeletal Defects

Metabolism Research

Mouse/Human Gene Homologs
mucopolysaccharidosis type VII, GUSB deficiency

Neurobiology Research
Behavioral and Learning Defects

Sensorineural Research

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)

Hofling AA; Vogler C; Creer MH; Sands MS. 2003. Engraftment of human CD34+ cells leads to widespread distribution of donor-derived cells and correction of tissue pathology in a novel murine xenotransplantation model of lysosomal storage disease. Blood 101(5):2054-63. [PubMed: 12406886]  [MGI Ref ID J:109848]

Additional References

Price and Supply Information

Strain Name: NOD.Cg-Prkdcscid Gusbmps/SndsJ
Stock Number: 005053

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 Mouse Mutant Resource 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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