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Strain Name:

CBySmn.CB17-Prkdcscid/J

Stock Number:

001803

Availability:

Level 2

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General Terms and Conditions

Former Name      BALB/c    (Changed: 15-DEC-04 )
Genes & Alleles   Prkdc;   Prkdcscid;

Product Information

Strain Details

Type JAX® GEMM® Strain - Congenic
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Mutant Strain
Mating SystemHomozygote x Homozygote         (Female x Male)
Specieslaboratory mouse
Background Strain BALB/cBySmn
Donor Strain C.BKa-Ighb/IcrSmn (C.B-17)
H2 Haplotyped
GenerationN7F60 (03-JAN-08)

Appearance
albino, affected
Related Genotype: Tyrp1b/Tyrp1b Tyrc/Tyrc Prkdcscid/Prkdcscid

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 C3H/HeJ background, and even lower on the NOD/ShiLtSz background. Note: BALB/cBy mice are Igh-1a while the original C.B-17 mice are Igh-1b.

Strain Development
Prkdcscid occurred spontaneously in a colony of BALB/c-Ighb (C.B-17) mice maintained at the Institute for Cancer Research in Philadelphia, PA. It was transferred to BALB/cByJSmn by Charles Sidman (Roths et al. and personal communication).

Related Disease (OMIM) Terms

Severe Combined Immunodeficiency, Autosomal Recessive, T Cell-Negative, B Cell-Negative, Nk Cell-Positive
Mammalian Phenotype Terms assigned by genotype

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Prkdcscid/Prkdcscid

        CB17-Prkdcscid
  • life span-post-weaning/aging
  • premature death (MGI Ref ID J:6958)
    • lifespan is shortened in conventional housing, however, in an SPF environment homozygotes can live to more than one year
  • immune system phenotype
  • *normal* immune system phenotype (MGI Ref ID J:22026)
    • in contrast to homozygotes on the NOD background, spleen cell suspensions from homozygous CB17 mice exhibit NK cell activity
    • abnormal humoral immune response (MGI Ref ID J:6958)
      • mice are unable to produce specific antibody to two T-independent antigens
      • decreased immunoglobulin level (MGI Ref ID J:6958)
        • 31/206 mice tested have low levels of serum immunoglobulin, all 31 have an IgG isotype and of these, 17 also have an IgM isotype
        • the remaining 175/206 mice do not have detectable serum immunoglobulin
        • only 1/11 homozygotescan produce greater than 1 ug/ml serum Ig at up to 100 days of age, however, 21/29 homozygotes produce greater than 1 ug/ml between 100-200 days of age
        • decreased IgG level (MGI Ref ID J:6958)
          • hypogammaglobulinemic
    • abnormal lymph node cellularity (MGI Ref ID J:6958)
      • lymph nodes have only a few lymphoid cells, however, sinuses are well-formed and contain macrophages
    • abnormal response to transplant (MGI Ref ID J:22026)
      • following injection of human T lymphoblastoid cells, 40% of nucleated spleen cells are of human origin by 4 weeks post injection
      • increased length of allograft survival (MGI Ref ID J:6958)
        • homozygotes do not reject full thickness skin allografts
        • 1/5 homozygotes (5-6 weeks of age) reject orthotopic tail skin allografts throughout a 3 month observation period
    • abnormal spleen white pulp morphology (MGI Ref ID J:6958)
      • splenic follicles are devoid of lymphocytic cells
    • abnormal thymus morphology (MGI Ref ID J:6958)
      • the remaining thymocytes that express the Thy1 marker are larger than normal thymocytes
      • abnormal thymus cortex morphology (MGI Ref ID J:6958)
        • lymphocytic cortex is not evident in thymus
        • mucinous cysts are occasionally found
      • small thymus (MGI Ref ID J:6958)
        • 1/10th to 1/100th normal size
    • absent Peyer's patches (MGI Ref ID J:6958)
      • PeyerŐs patches are rarely visible
    • decreased B cell proliferation (MGI Ref ID J:6958)
      • spleen cells do not proliferate in response to LPS
    • decreased T cell proliferation (MGI Ref ID J:6958)
      • splenic T cells do not proliferate in response to concanavalin A or to a one-way mixed lymphocyte reaction
    • decreased leukocyte cell number (MGI Ref ID J:6958)
      • homozygotes are leukopenic
      • absent B cells (MGI Ref ID J:6958)
        • B cells cannot be detected in the spleen with Ig or Lyb-8 (Cd22) markers
        • absent pre-B cells (MGI Ref ID J:6958)
          • pre-B cells cannot be detected in the bone marrow
    • salivary gland inflammation (MGI Ref ID J:21965)
      • in mice injected i.p. with cells from submandibular gland tissue of diseased Faslpr mice, inflammatory lesions are observed in salivary and lacrimal glands
      • infiltrating cells are CD4+ and Vbeta8+ with a minority being CD4+ and Vbeta6+
      • mice injected with cells pretreated with anti-CD4 or anti-Vbeta8 do not develop inflammatory lesions or only minor lesions are seen in parotid, submandibular, sublingual and lacrimal glands in majority of transplanted mice
    • small lymphoid organs (MGI Ref ID J:6958)
      • lymphoid hypoplasia (MGI Ref ID J:6958)
        • lymphid organs are 1/10th or less of normal size
        • lymph organs consist mostly of supportive tissue with varying numbers of fibroblasts, macrophages and histiocytes
  • hematopoietic system phenotype
  • *normal* hematopoietic system phenotype (MGI Ref ID J:22026)
    • in contrast to homozygotes on the NOD background, homozygous CB17 mice exhibit normal erythrocyte mean cell volumes
    • abnormal spleen white pulp morphology (MGI Ref ID J:6958)
      • splenic follicles are devoid of lymphocytic cells
    • abnormal thymus morphology (MGI Ref ID J:6958)
      • the remaining thymocytes that express the Thy1 marker are larger than normal thymocytes
      • abnormal thymus cortex morphology (MGI Ref ID J:6958)
        • lymphocytic cortex is not evident in thymus
        • mucinous cysts are occasionally found
      • small thymus (MGI Ref ID J:6958)
        • 1/10th to 1/100th normal size
    • decreased B cell proliferation (MGI Ref ID J:6958)
      • spleen cells do not proliferate in response to LPS
    • decreased T cell proliferation (MGI Ref ID J:6958)
      • splenic T cells do not proliferate in response to concanavalin A or to a one-way mixed lymphocyte reaction
    • decreased leukocyte cell number (MGI Ref ID J:6958)
      • homozygotes are leukopenic
      • absent B cells (MGI Ref ID J:6958)
        • B cells cannot be detected in the spleen with Ig or Lyb-8 (Cd22) markers
        • absent pre-B cells (MGI Ref ID J:6958)
          • pre-B cells cannot be detected in the bone marrow
  • tumorigenesis
  • T cell derived lymphoma (MGI Ref ID J:6958)
    • spontaneous T cell lymphomas are found in greater than 10% of homozygotes at 5-9 months of age
    • tumors arise in the thymus and are highly invasive and are transplantable
  • thymic lymphoma (MGI Ref ID J:6958)
  • digestive/alimentary phenotype
  • salivary gland inflammation (MGI Ref ID J:21965)
    • in mice injected i.p. with cells from submandibular gland tissue of diseased Faslpr mice, inflammatory lesions are observed in salivary and lacrimal glands
    • infiltrating cells are CD4+ and Vbeta8+ with a minority being CD4+ and Vbeta6+
    • mice injected with cells pretreated with anti-CD4 or anti-Vbeta8 do not develop inflammatory lesions or only minor lesions are seen in parotid, submandibular, sublingual and lacrimal glands in majority of transplanted mice
  • endocrine/exocrine gland phenotype
  • salivary gland inflammation (MGI Ref ID J:21965)
    • in mice injected i.p. with cells from submandibular gland tissue of diseased Faslpr mice, inflammatory lesions are observed in salivary and lacrimal glands
    • infiltrating cells are CD4+ and Vbeta8+ with a minority being CD4+ and Vbeta6+
    • mice injected with cells pretreated with anti-CD4 or anti-Vbeta8 do not develop inflammatory lesions or only minor lesions are seen in parotid, submandibular, sublingual and lacrimal glands in majority of transplanted mice

Prkdcscid/Prkdcscid

        B6.CB17-Prkdcscid/Sz
  • immune system phenotype
  • abnormal NK cell physiology (MGI Ref ID J:34814)
    • NK cell activity is markedly elevated in comparison to control
  • abnormal Peyer's patch morphology (MGI Ref ID J:34814)
    • patches contain few lymphoid cells
    • small Peyer's patches (MGI Ref ID J:34814)
  • abnormal complement physiology (MGI Ref ID J:34814)
    • homozygotes exhibit elevated levels of complement activity in comparison to control
  • abnormal level of surface class II molecules (MGI Ref ID J:34814)
    • class II expression is reduced 4 fold on spleen cells in 10-14 week old mice
  • abnormal lymph node morphology (MGI Ref ID J:34814)
    • follicles are absent
  • abnormal spleen white pulp morphology (MGI Ref ID J:34814)
    • follicles are absent
  • abnormal thymus cellularity (MGI Ref ID J:34814)
    • thymus consists of reticular tissues and some cysts
  • decreased immunoglobulin level (MGI Ref ID J:34814)
    • only 2/10 homozygotes produce greater than 1 ug/ml serum Ig between 1-29 weeks of age, however, between 30-57 weeks of age all mice produce greater than 1 ug/ml (ranging from 2-30 ug/ml)
  • decreased lymphocyte cell number (MGI Ref ID J:34814)
    • percentages of circulating lymphocytes are significantly decreased in comparison to control
    • decreased B cell number (MGI Ref ID J:34814)
      • B220+ splenic B cells are decreased (53.0.0% vs. 4.7%) in comparison to C57BL/6 control in 10-14 week old mice
      • decreased pre-B cell number (MGI Ref ID J:34814)
        • there is a significant absence of splenic B cells bearing the Ig kappa light chain (40.0% vs. 0.9%) in 10-14 week old mice
    • decreased T cell number (MGI Ref ID J:34814)
      • significant numbers of CD3+ splenic T cells are absent in comparison to control (37.1% vs. <0.1%) in 10-14 week old mice
      • decreased CD4-positive T cell number (MGI Ref ID J:34814)
        • in addition, existing CD4+ cells are not CD3+CD4+
      • decreased CD8-positive T cell number (MGI Ref ID J:34814)
        • in addition, existing CD8+ cells are not CD3+CD8+
  • increased NK cell number (MGI Ref ID J:34814)
    • NK1.1+ splenic cells are increased by 8 fold in comparison to control in 10-14 week old mice, however, numbers are reduced in 10-12 month old mice
  • increased granulocyte number (MGI Ref ID J:34814)
    • Gr1+ splenic and bone marrow granulocytes are increased by 4 fold in comparison to control in 10-14 week old mice and continue increasing with age
    • increased eosinophil cell number (MGI Ref ID J:34814)
    • increased neutrophil cell number (MGI Ref ID J:34814)
  • increased macrophage cell number (MGI Ref ID J:34814)
    • F4/80+ splenic macrophages are increased by almost 7 fold in comparison to control in 10-14 week old mice, however numbers are reduced in 10-12 month old mice
  • increased monocyte cell number (MGI Ref ID J:34814)
  • small thymus (MGI Ref ID J:34814)
  • spleen hypoplasia (MGI Ref ID J:34814)
    • homozygotes have four fold reduction in spleen cellularity at 10-14 weeks old as compared to controls
    • mice aged to 10-12 months have some increase in cellularity, but it remains less than half that of control mice
  • stomach inflammation (MGI Ref ID J:120556)
    • 5 weeks after Helicobacter pylori infection, mice have a significantly decreased gastritis inflammation score compared to infected wild-type mice
  • hematopoietic system phenotype
  • abnormal spleen white pulp morphology (MGI Ref ID J:34814)
    • follicles are absent
  • abnormal thymus cellularity (MGI Ref ID J:34814)
    • thymus consists of reticular tissues and some cysts
  • decreased lymphocyte cell number (MGI Ref ID J:34814)
    • percentages of circulating lymphocytes are significantly decreased in comparison to control
    • decreased B cell number (MGI Ref ID J:34814)
      • B220+ splenic B cells are decreased (53.0.0% vs. 4.7%) in comparison to C57BL/6 control in 10-14 week old mice
      • decreased pre-B cell number (MGI Ref ID J:34814)
        • there is a significant absence of splenic B cells bearing the Ig kappa light chain (40.0% vs. 0.9%) in 10-14 week old mice
    • decreased T cell number (MGI Ref ID J:34814)
      • significant numbers of CD3+ splenic T cells are absent in comparison to control (37.1% vs. <0.1%) in 10-14 week old mice
      • decreased CD4-positive T cell number (MGI Ref ID J:34814)
        • in addition, existing CD4+ cells are not CD3+CD4+
      • decreased CD8-positive T cell number (MGI Ref ID J:34814)
        • in addition, existing CD8+ cells are not CD3+CD8+
  • increased NK cell number (MGI Ref ID J:34814)
    • NK1.1+ splenic cells are increased by 8 fold in comparison to control in 10-14 week old mice, however, numbers are reduced in 10-12 month old mice
  • increased granulocyte number (MGI Ref ID J:34814)
    • Gr1+ splenic and bone marrow granulocytes are increased by 4 fold in comparison to control in 10-14 week old mice and continue increasing with age
    • increased eosinophil cell number (MGI Ref ID J:34814)
    • increased neutrophil cell number (MGI Ref ID J:34814)
  • increased macrophage cell number (MGI Ref ID J:34814)
    • F4/80+ splenic macrophages are increased by almost 7 fold in comparison to control in 10-14 week old mice, however numbers are reduced in 10-12 month old mice
  • increased monocyte cell number (MGI Ref ID J:34814)
  • small thymus (MGI Ref ID J:34814)
  • spleen hypoplasia (MGI Ref ID J:34814)
    • homozygotes have four fold reduction in spleen cellularity at 10-14 weeks old as compared to controls
    • mice aged to 10-12 months have some increase in cellularity, but it remains less than half that of control mice
  • digestive/alimentary phenotype
  • stomach inflammation (MGI Ref ID J:120556)
    • 5 weeks after Helicobacter pylori infection, mice have a significantly decreased gastritis inflammation score compared to infected wild-type mice

Prkdcscid/Prkdcscid

        involves: BALB/c * CB17
  • digestive/alimentary phenotype
  • abnormal intestinal epithelium morphology (MGI Ref ID J:126867)
    • migration of proliferated intestinal epithelial cells (IEC) toward the top of the villi, the number of proliferating IECs, and MHC class II expression on IECs are augmented in mutants compared to wild-type controls

Gene & Allele Details

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

  Allele   Control
 Prkdcscid  001026 BALB/cByJ
 
  Considerations for Choosing Controls

Genotyping Protocols

Prkdcscid

Colony Maintenance

Diet Information JAX West and Bar Harbor Diets

Related Strains

View Strains carrying   Prkdcscid     (25 strains)

Additional Web Information

Congenic Nomenclature
Genetic Quality Control Annual Report
JAX® NOTES, Spring 1993; 453. The Severe Combined Immunodeficiency (scid) Mutation.
JAX® NOTES, Spring 1997; 469. Helicobacter Infections in Laboratory Mice.
JAX® NOTES, Spring 2006; 501. Choosing an Immunodeficient Mouse Model.

Animal Health Reports

Room Number           AX30
Room Number           RB07

Research Applications

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

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

Blunt T; Finnie NJ; Taccioli GE; Smith GC; Demengeot J; Gottlieb TM; Mizuta R; Varghese AJ; Alt FW; Jeggo PA; Jackson SP. 1995. Defective DNA-dependent protein kinase activity is linked to V(D)J recombination and DNA repair defects associated with the murine scid mutation. Cell 80(5):813-23. [PubMed: 7889575]  [MGI Ref ID J:30365]

Blunt T; Gell D; Fox M; Taccioli GE; Lehmann AR; Jackson SP; Jeggo PA. 1996. Identification of a nonsense mutation in the carboxyl-terminal region of DNA-dependent protein kinase catalytic subunit in the scid mouse. Proc Natl Acad Sci U S A 93(19):10285-90. [PubMed: 8816792]  [MGI Ref ID J:35393]

Bosma M; Schuler W; Bosma G. 1988. The scid mouse mutant. Curr Top Microbiol Immunol 137:197-202. [PubMed: 3416632]  [MGI Ref ID J:9341]

Custer RP; Bosma GC; Bosma MJ. 1985. Severe combined immunodeficiency (SCID) in the mouse. Pathology, reconstitution, neoplasms. Am J Pathol 120(3):464-77. [PubMed: 2412448]  [MGI Ref ID J:30980]

Dorshkind K; Keller GM; Phillips RA; Miller RG; Bosma GC; O'Toole M; Bosma MJ. 1984. Functional status of cells from lymphoid and myeloid tissues in mice with severe combined immunodeficiency disease. J Immunol 132(4):1804-8. [PubMed: 6607948]  [MGI Ref ID J:7343]

Nonoyama S; Smith FO; Bernstein ID; Ochs HD. 1993. Strain-dependent leakiness of mice with severe combined immune deficiency. J Immunol 150(9):3817-24. [PubMed: 8473734]  [MGI Ref ID J:4610]

Roths JB; Smith AL; Sidman CL. 1993. Lethal exacerbation of Pneumocystis carinii pneumonia in severe combined immunodeficiency mice after infection by pneumonia virus of mice. J Exp Med 177(4):1193-8. [PubMed: 8459214]  [MGI Ref ID J:109921]

Additional References

Price and Supply Information

Strain Name: CBySmn.CB17-Prkdcscid/J
Stock Number: 001803

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Standard SupplyLevel 2. Up to 100 mice. Larger quantities or custom orders arranged upon request.
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.
Genomic DNA is available for this strain from the Mouse DNA Resource.
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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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