Overview

Also Known As:NSG hprt-null

These mutant mice combine the immunological characteristics of the NOD/ShiLtJ background, the severe combined immune deficiency mutation (scid), IL2 receptor gamma chain deficiency and a hypoxanthine phosphoribosyl transferase (Hprt^b-m3) null allele. This mutant mouse strain may be useful for xenotransplantation and drug screening.

Donating Investigator

James Eshleman, John Hopkins University

Genetic overview

Genetic Background	Generation

Prkdc^scid

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

Il2rg^tm1Wjl

Allele Type	Gene Symbol	Gene Name
Targeted (Null/Knockout)	Il2rg	interleukin 2 receptor, gamma chain

Hprt^b-m3

Allele Type	Gene Symbol	Gene Name
Spontaneous	Hprt	hypoxanthine guanine phosphoribosyl transferase

View Genetics

Research Applications

Virology Research
Cancer Research
Immunology, Inflammation and Autoimmunity Research
Research Tools
Internal/Organ Research
Metabolism Research
Neurobiology Research

View All Research Applications

Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

View Price List

Details

Detailed Description

Mice that are homozygous/hemizygous for these alleles are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. These mutant mice combine the following characteristics: NK cell deficiency (NOD/ShiLtJ background), T and B cell deficiency (Prkdc^scid), reduced numbers of lymphocytes and myeloid dendritic cells (Il2rg^tm1Wjl) and biochemically defective stromal cells (Hprt^bm-3). These mice can be used for transplantation of human primary cancers. During tumor growth, Hprt-defective murine fibroblast and stromal cells replace human stromal cells. In culture, the addition of HAT selection media eliminates murine fibroblasts and other stromal cells and facilitates the isolation of human cancer cells. These mice may be used to generate patient-specific low passage cell lines from primary cancers for use in chemosensitivity profiling and other applications.

Development

The Hprt^bm-3 allele, a spontaneous 36 kb deletion at the 3' end of exon 2, was selected from an HPRT deficient 129P2/OlaHsd-derived E14TG2a embryonic stem cell line (ES). The X-linked mutation was introgressed into C57BL/6 mice for at least 5 generations. A heterozygous female was crossed to a NOD.Cg-Prkdc^scid Il2rg^tm1Wjl/SzJ (Stock No. 005557) male; the backcross was continued for five generations. Correctly genotyped pups from each generation were selected using marker assisted genome scan analysis (speed congenic).

Control Suggestions

Additional Information

Considerations for Choosing Controls

Selected References

Kamiyama H; Rauenzahn S; Shim JS; Karikari CA; Feldmann G; Hua L; Kamiyama M; Schuler FW; Lin MT; Beaty RM; Karanam B; Liang H; Mullendore ME; Mo G; Hidalgo M; Jaffee E; Hruban RH; Jinnah HA; Roden RB; Jimeno A; Liu JO; Maitra A; Eshleman JR. 2013. Personalized chemotherapy profiling using cancer cell lines from selectable mice. Clin Cancer Res 19(5):1139-46PubMed: 23340293 MGI: J:202087
McEwan C; Melton DW. 2003. A simple genotyping assay for the Hprt null allele in mice produced from the HM-1 and E14TG2a mouse embryonic stem cell lines. Transgenic Res 12(4):519-20PubMed: 12885172 MGI: J:159603

View All References

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*).

Il2rg^tm1Wjl

Allele Symbol: Il2rg^tm1Wjl

Allele Name	targeted mutation 1, Warren J Leonard
Allele Type	Targeted (Null/Knockout)
Allele Synonym(s)	[KO]gammac; CD132^-; gammac^-; gc^-; Il2rg^tm1Wjll; IL2Rgamma^null
Gene Symbol and Name	Il2rg, interleukin 2 receptor, gamma chain
Gene Synonym(s)
Site of Expression	Primarily lymphoid cells.
Strain of Origin	129S4/SvJae
Chromosome	X
Molecular Note	A neomycin resistance cassette replaced part of exon 3 and all of exons 4 - 8 of the gene, resulting in the loss of most of the extracellular domain and all of the transmembrane and cytoplasmic domains of the protein.
Mutations Made By	Dr. Warren Leonard, NHLBI, NIH

Hprt^b-m3

Allele Symbol: Hprt^b-m3

Allele Name	hypoxanthine guanine phosphoribosyl transferase B, mutation 3
Allele Type	Spontaneous
Allele Synonym(s)	Hprt^-
Gene Symbol and Name	Hprt, hypoxanthine guanine phosphoribosyl transferase
Gene Synonym(s)
Strain of Origin	129P2/OlaHsd
Chromosome	X
General Note	HPRT- embryonic stem cells were obtained by selecting for spontaneous mutation by incubation in medium containing 6-thioguanine. HPRT- males have no overt phenotype of abnormal behavior (J:15483). The mutation is due to a large deletion in the Hprt gene. In situ hybridization studies showed HPRT mRNA in high levels in most neurons, but not in glial cells, in normal mice. No HPRT mRNA was detected in the brains of male mice carrying this deletion (J:2058). Mutant mice have no HPRT detectable by Western blot analysis and no detectable HPRT enzyme activity in brain homogenates. They appear to have normal brain purine content, but de novo purine synthesis is accelerated four- to fivefold (J:11842). The Hprt^b-m3 mutation has been used in preimplantation studies to determine when the maternal and paternal alleles of Hprt are activated during early embryonic development (J:2389). Either administration of amphetamine (J:1847) or inhibition of adenine phosphoribosyltransferase (APRT) activity (J:4123) stimulates locomotor and stereotypic behaviors in HPRT-deficient mice. However, the null mutant for both Hprt and Aprt does not show the characteristics of Lesch-Nyhan disease (J:35822).Cells from mice hemizygous or homozygous for this mutation are HPRT-deficient and resistant to the drug 6-thioguanine (6TG).
Molecular Note	The allele contains a ~55 kb deletion spanning the promoter and first 2 exons. Subsequent direct sequence comparison with wild type DNA defined the exact breakpoints of the deletion, which lies 415 bp after the 3' end of exon 2, and determined the deletion size to be 36 kb.
Mutations Made By	Elizabeth Simpson, Centre for Molecular Medicine & Therapeutics, University of British Columbia

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Genotype: Prkdc^scid related

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
Immunology, Inflammation and Autoimmunity Research
- Immunodeficiency
  - B and T cell deficiency

Genotype: Il2rg^tm1Wjl related

Cancer Research
- Growth Factors/Receptors/Cytokines
Immunology, Inflammation and Autoimmunity Research
- Growth Factors/Receptors/Cytokines

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

Genotype: Hprt^b-m3 related

Metabolism Research
Neurobiology Research
- Neurodegeneration

Mammalian Phenotype Terms by Genotype

References

Kamiyama H; Rauenzahn S; Shim JS; Karikari CA; Feldmann G; Hua L; Kamiyama M; Schuler FW; Lin MT; Beaty RM; Karanam B; Liang H; Mullendore ME; Mo G; Hidalgo M; Jaffee E; Hruban RH; Jinnah HA; Roden RB; Jimeno A; Liu JO; Maitra A; Eshleman JR. 2013. Personalized chemotherapy profiling using cancer cell lines from selectable mice. Clin Cancer Res 19(5):1139-46PubMed: 23340293 MGI: J:202087
McEwan C; Melton DW. 2003. A simple genotyping assay for the Hprt null allele in mice produced from the HM-1 and E14TG2a mouse embryonic stem cell lines. Transgenic Res 12(4):519-20PubMed: 12885172 MGI: J:159603

Additional - Hprt^b-m3 related

Additional - Il2rg^tm1Wjl related

Additional - Prkdc^scid related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Standard PCR:Hprt
- Probe:Il2rg-PROBE
- End Point Analysis:Prkdc End Point
- Genotyping resources and troubleshooting
Breeding Considerations

When maintaining a live colony, these mice are bred as homozygous for the Prkdc^scid allele and homozygous/hemizygous for the X-linked Il2rg^tm1Wjl and Hprt^b-m3 alleles. SPF (specific pathogen-free) conditions are recommended.
- Additional Breeding and Husbandry Support
Citation
When using the NSG hprt-null mouse strain in a publication, please cite the originating article(s) and include JAX stock #012480 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200

Pricing & Availability

Cryo Recovery

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Not-For-Profit & Academic Pricing

Service/Product	Description	Price
	Homozygous for Prkdc<scid>, X linked Homozygous females and Hemizygous Males for Il2rg<tm1Wjl>, X linked Homozygous females and Hemizygous Males for Hprt<b-m3>/EshJ, 1 pair minimum

Related Products and Services

Frozen Mouse Embryo	NOD.Cg-Prkdc<scid> Il2rg<tm1Wjl> Hprt<b-m3>/ Frozen Embryos	$2595.00
Frozen Mouse Embryo	NOD.Cg-Prkdc<scid> Il2rg<tm1Wjl> Hprt<b-m3>/ Frozen Embryos	$2595.00
Frozen Mouse Embryo	NOD.Cg-Prkdc<scid> Il2rg<tm1Wjl> Hprt<b-m3>/ Frozen Embryos	$3373.50
Frozen Mouse Embryo	NOD.Cg-Prkdc<scid> Il2rg<tm1Wjl> Hprt<b-m3>/ Frozen Embryos	$3373.50

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The Jackson Laboratory's Genotype Promise

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. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.

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General Terms and Conditions

Questions about Terms of Use

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Licensing Information

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Also Known As:NSG hprt-null

Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Control Suggestions

Additional Information

Selected References

Prkdcscid

Allele Symbol: Prkdcscid

Il2rgtm1Wjl

Allele Symbol: Il2rgtm1Wjl

Hprtb-m3

Allele Symbol: Hprtb-m3

Disease Terms

Research Areas By Phenotype

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

Genotype: Prkdcscid related

Genotype: Il2rgtm1Wjl related

Genotype: Hprtb-m3 related

Mammalian Phenotype Terms by Genotype

References

Additional - Hprtb-m3 related

Additional - Il2rgtm1Wjl related

Additional - Prkdcscid related

Genotyping Protocols

Breeding Considerations

Citation

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200

Live Mouse

Cryorecovery - Not-For-Profit & Academic Pricing

Related Products and Services

Payment Terms and Conditions

The Jackson Laboratory's Genotype Promise

Terms Of Use

Additional Use Restrictions Apply

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

Il2rg^tm1Wjl

Allele Symbol: Il2rg^tm1Wjl

Hprt^b-m3

Allele Symbol: Hprt^b-m3

Genotype: Prkdc^scid related

Genotype: Il2rg^tm1Wjl related

Genotype: Hprt^b-m3 related

Additional - Hprt^b-m3 related

Additional - Il2rg^tm1Wjl related

Additional - Prkdc^scid related