Overview

This strain has been discontinued, please refer to Stock No. 010816

Genetic overview

Genetic Background	Generation

Prkdc^scid

Allele Type	Gene Symbol	Gene Name
Spontaneous	Prkdc	protein kinase, DNA activated, catalytic polypeptide

Allele Type	Gene Symbol	Gene Name
Spontaneous	Ghrhr	growth hormone releasing hormone receptor

Research Applications

Immunology, Inflammation and Autoimmunity Research
Virology Research
Developmental Biology Research
Endocrine Deficiency Research
Mouse/Human Gene Homologs
Research Tools
Internal/Organ Research

View All Research Applications

Details

Detailed Description

Mice homozygous for the severe combined immune deficiency spontaneous mutation (Prkdc^scid, 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. In addition to the effects of the scid mutation, Ghrhr^litPrkdc^scid /Bm mice are deficient in growth hormone and IGF1 while Ghrh^hpgPrkdc^scid/Bm mice (Stock No. 002038) are deficient in gonadotropins and gonodal steroids. These two double mutant strains are useful for determining endocrine dependence of grafted cells and tissues.

Development

C57BL/6J-Ghrhr^lit/Ghrhr^lit x C.B-17-Prkdc^scid/Prkdc^scid offspring were sibling mated. The strain was made homozygous for Prkdc^scid and kept segregating for Ghrhr^lit and the sibling mating continued to the present.

Control Suggestions

Heterozygote from the colony
000664 C57BL/6J

Additional Information

Considerations for Choosing Controls

Genetics

Prkdc^scid

Allele Symbol: Prkdc^scid

Allele Name	severe combined immunodeficiency
Allele Type	Spontaneous
Allele Synonym(s)	SCID
Gene Symbol and Name	Prkdc, protein kinase, DNA activated, catalytic polypeptide
Gene Synonym(s)
Site of Expression	T and B lymphocytes.
Strain of Origin	C.BKa-Igh^b/Icr
Chromosome	16
Molecular Note	A T-to-A transversion point mutation at a position corresponding to codon 4046 (codon 4095 in transcript ENSMUST00000023352.8) created a premature stop codon (p.Y4046*).

Ghrhr^lit

Allele Symbol: Ghrhr^lit

Allele Name	little
Allele Type	Spontaneous
Allele Synonym(s)	lit; lit^m
Gene Symbol and Name	Ghrhr, growth hormone releasing hormone receptor
Gene Synonym(s)
Strain of Origin	C57BL/6J
Chromosome	6
Molecular Note	The mutation in little mice is a A-to-G transition in codon 60 that alters this residue from a aspartate to a glycine in the encoded protein (p.D60G). Reduced levels of mRNA were noted in total RNA derived from pituitary of homozygous mice, and in vitro assays demonstrated that no functional protein is made from this allele.

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Genotype: Prkdc^scid related

Immunology, Inflammation and Autoimmunity Research
- Immunodeficiency
  - B and T cell deficiency
Virology Research
- B and T Cell Deficiency
  - AIDS research tool
Research Tools
- Toxicology Research
  - xenograft/transplant host
- Cancer Research
  - B and T cell deficiency, xenograft/transplant host
Internal/Organ Research
- Lymphoid Tissue Defects
  - B and T cell deficiency

Genotype: Ghrhr^lit related

Developmental Biology Research
- Growth Defects
Endocrine Deficiency Research
- Hypothalamus/Pituitary Defects
Mouse/Human Gene Homologs
- growth hormone deficiency

Research Tools
- Immunology, Inflammation and Autoimmunity Research
  - B and T cell deficiency
- Cancer Research
  - xenograft/transplant host

Mammalian Phenotype Terms by Genotype

References

Additional References

Beamer WG; Shultz KL; Tennent BJ; Shultz LD. 1993. Granulosa cell tumorigenesis in genetically hypogonadal-immunodeficient mice grafted with ovaries from tumor-susceptible donors. Cancer Res 53(16):3741-6PubMed: 8339285 MGI: J:14443
Bosma M; Schuler W; Bosma G. 1988. The scid mouse mutant. Curr Top Microbiol Immunol 137:197-202PubMed: 3416632 MGI: J:9341
Custer RP; Bosma GC; Bosma MJ. 1985. Severe combined immunodeficiency (SCID) in the mouse. Pathology, reconstitution, neoplasms. Am J Pathol 120(3):464-77PubMed: 2412448 MGI: J:30980
Godfrey P; Rahal JO; Beamer WG; Copeland NG; Jenkins NA; Mayo KE. 1993. GHRH receptor of little mice contains a missense mutation in the extracellular domain that disrupts receptor function. Nat Genet 4(3):227-32PubMed: 8395283 MGI: J:12846
Lin SC; Lin CR; Gukovsky I; Lusis AJ; Sawchenko PE; Rosenfeld MG. 1993. Molecular basis of the little mouse phenotype and implications for cell type-specific growth [see comments] Nature 364(6434):208-13PubMed: 8391647 MGI: J:13404
Many MC; Drexhage HA; Denef JF. 1993. High frequency of thymic ectopy in thyroids from autoimmune prone nonobese diabetic female mice. Lab Invest 69(3):364-7PubMed: 8377477 MGI: J:14747
Nonoyama S; Smith FO; Bernstein ID; Ochs HD. 1993. Strain-dependent leakiness of mice with severe combined immune deficiency. J Immunol 150(9):3817-24PubMed: 8473734 MGI: J:4610
Prochazka M; Gaskins HR; Shultz LD; Leiter EH. 1992. The nonobese diabetic scid mouse: model for spontaneous thymomagenesis associated with immunodeficiency. Proc Natl Acad Sci U S A 89(8):3290-4PubMed: 1373493 MGI: J:513
Serreze DV; Leiter EH; Hanson MS; Christianson SW; Shultz LD; Hesselton RM; Greiner DL. 1995. Emv30null NOD-scid mice. An improved host for adoptive transfer of autoimmune diabetes and growth of human lymphohematopoietic cells. Diabetes 44(12):1392-8PubMed: 7589844 MGI: J:29951
Shultz LD; Schweitzer PA; Christianson SW; Gott B; Schweitzer IB; Tennent B; McKenna S; Mobraaten L; Rajan TV; Greiner DL; Leiter EH. 1995. Multiple defects in innate and adaptive immunologic function in NOD/LtSz-scid mice. J Immunol 154(1):180-91PubMed: 7995938 MGI: J:22026

Additional - Ghrhr^lit related

Additional - Prkdc^scid related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Genotyping resources and troubleshooting

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Questions about Terms of Use

Licensing Information

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Genetic overview

Research Applications

Detailed Description

Development

Control Suggestions

Additional Information

Prkdcscid

Allele Symbol: Prkdcscid

Ghrhrlit

Allele Symbol: Ghrhrlit

Disease Terms

Research Areas By Phenotype

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

Genotype: Prkdcscid related

Genotype: Ghrhrlit related

Mammalian Phenotype Terms by Genotype

References

Additional References

Additional - Ghrhrlit related

Additional - Prkdcscid related

Genotyping Protocols

Terms Of Use

Licensing Information

JAX® Mice, Products & Services Conditions of Use

No Warranty

Credit for PRODUCTS or SERVICES

Animal Care and Use for SERVICES

No Liability

Prkdc^scid

Allele Symbol: Prkdc^scid

Ghrhr^lit

Allele Symbol: Ghrhr^lit

Genotype: Prkdc^scid related

Genotype: Ghrhr^lit related

Additional - Ghrhr^lit related

Additional - Prkdc^scid related