Overview

Also Known As:NSG-PiZ

NSG-PiZ mice express mutant human SERPINA1 (Glu342Lys mutation) on the immunodeficient NOD scid gamma (NSG) background, providing a stable engraftment environment for primary human hepatocytes after partial hepatectomy. This strain may be useful in studies utilizing a humanized liver xenograft mouse model.

Donating Investigator

Dr. Leonard D. Shultz, The Jackson Laboratory

Genetic overview

Genetic Background	Generation
	Contact Technical Support (2021-02-08 00:00:00)

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

Tg(SERPINA1*E342K)#Slcw

Allele Type
Transgenic (Inserted expressed sequence, Humanized sequence)

View Genetics

Research Applications

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

View All Research Applications

Base Price

Starting at:

$250.92 Domestic price for male 3-week

View Price List

Details

Detailed Description

NSG-PiZ mice do not express the Prkdc gene nor the X-linked Il2rg gene, but do express the mutant human SERPINA1 PiZ variant with a Glu342Lys point mutation. Female NSG-PiZ mice, homozygous for both alleles and the transgene, and male NSG-PiZ mice, homozygous for the Prkdc allele and transgene and hemizygous for the X linked Il2rg allele, are viable and fertile. After partial (two-thirds) hepatectomy, NSG-PiZ mice engrafted with primary human hepatocytes exhibit stable human albumin serum levels between 0.4 and 2.8 mg/ml.

Development

Transgenic Tg(SERPINA1*E342K)#Slcw mice on a segregating background were obtained by Dr. Leonard Shultz (The Jackson Laboratory) from Dr. David Perlmutter (University of Pittsburgh School of Medicine). The mice were crossed to NOD scid females (Stock No. 001303), and then backcrossed to NSG, NOD.Cg-Prkdc^scid Il2rg^tm1Wjl/SzJ, (Stock No. 005557) mice using a marker assisted speed congenic protocol.

The Tg(SERPINA1*E342K)#Slcw transgenic construct containing the entire PiZ variant of the human SERPINA1, serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1, gene was injected into fertilized (ICR x C57BL/6)F1 mouse eggs. The mice may have been crossed to FVB/N.

NSG double mutant mice were produced by breeding female NOD.CB17-Prkdc^scid/J (Stock No. 001303) mice with male mice bearing the X-linked B6.129S4-Il2rg^tm1Wjl/J allele (Stock No. 003174). The resulting male mice heterozygous for the Prkdc^scid allele and hemizygous for the Il2rg^tm1Wjl allele were crossed to female NOD.CB17-Prkdc^scid/J (Stock No. 001303) mice for eight generations. Heterozygotes were interbred to produce mice homozygous for the Prkdc^scid allele and homozygous (females) or hemizygous (males) for the Il2rg^tm1Wjl allele.

Expression Data

Expressed Gene	SERPINA1, serpin family A member 1, human
Site of Expression

Control Suggestions

Approximate Controls

005557 NOD.Cg-Prkdc^scid Il2rg^tm1Wjl/SzJ

Additional Information

Considerations for Choosing Controls

Selected References

Borel F; Tang Q; Gernoux G; Greer C; Wang Z; Barzel A; Kay MA; Shultz LD; Greiner DL; Flotte TR; Brehm MA; Mueller C. 2017. Survival Advantage of Both Human Hepatocyte Xenografts and Genome-Edited Hepatocytes for Treatment of alpha-1 Antitrypsin Deficiency. Mol Ther 25(11):2477-2489PubMed: 29032169 MGI: J:243726
Li S; Ling C; Zhong L; Li M; Su Q; He R; Tang Q; Greiner DL; Shultz LD; Brehm MA; Flotte TR; Mueller C; Srivastava A; Gao G. 2015. Efficient and Targeted Transduction of Nonhuman Primate Liver With Systemically Delivered Optimized AAV3B Vectors. Mol Ther 23(12):1867-76PubMed: 26403887 MGI: J:230567

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

Tg(SERPINA1*E342K)#Slcw

Allele Symbol: Tg(SERPINA1*E342K)#Slcw

Allele Name	transgene insertion, Savio L C Woo
Allele Type	Transgenic (Inserted expressed sequence, Humanized sequence)
Allele Synonym(s)	ATZ; PiZ; Tg(SERPINA1*E342K)Z1.01Slcw; Z1.01
Gene Symbol and Name	Tg(SERPINA1*E342K)#Slcw, transgene insertion, Savio L C Woo
Gene Synonym(s)
Promoter	SERPINA1, serpin family A member 1, human
Expressed Gene	SERPINA1, serpin family A member 1, human
Strain of Origin	(ICR x C57BL/6)F1
Chromosome	UN
Molecular Note	The transgene construct contains the entire human alpha₁-antitrypsin (AAT or SERPINA1) gene encoding the Z variant allele which has a Glu->Lys substitution at position 342, plus 2.0 and 2.3 kb of 5' and 3' genomic sequences, respectively.

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Genotype: Prkdc^scid related

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
Virology Research
- B and T Cell Deficiency
  - AIDS research tool

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

Genotype: Il2rg^tm1Wjl related

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

Mammalian Phenotype Terms by Genotype

References

Borel F; Tang Q; Gernoux G; Greer C; Wang Z; Barzel A; Kay MA; Shultz LD; Greiner DL; Flotte TR; Brehm MA; Mueller C. 2017. Survival Advantage of Both Human Hepatocyte Xenografts and Genome-Edited Hepatocytes for Treatment of alpha-1 Antitrypsin Deficiency. Mol Ther 25(11):2477-2489PubMed: 29032169 MGI: J:243726
Li S; Ling C; Zhong L; Li M; Su Q; He R; Tang Q; Greiner DL; Shultz LD; Brehm MA; Flotte TR; Mueller C; Srivastava A; Gao G. 2015. Efficient and Targeted Transduction of Nonhuman Primate Liver With Systemically Delivered Optimized AAV3B Vectors. Mol Ther 23(12):1867-76PubMed: 26403887 MGI: J:230567

Additional - Il2rg^tm1Wjl related

Additional - Prkdc^scid related

Additional - Tg(SERPINA1*E342K)#Slcw related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Probe:Il2rg-PROBE
- End Point Analysis:Prkdc End Point
- QPCR:Tg(SERPINA1*E342K)#Slcw
- Genotyping resources and troubleshooting
Breeding Considerations

This strain is a challenging breeder.

When maintaining a live colony, females that are homozygous for the Prkdc^scid and Il2rg^tm1Wjl alleles and the SERPINA1* transgene are bred to males that are homozygous for Prkdc^scid and SERPINA1* transgene, and hemizygous for Il2rg^tm1Wjl (Il2rg is an X-linked gene).
- Additional Breeding and Husbandry Support
Mating System
- Homozygous Prkdc Homozygous Il2rgHomozygous Tg(SERPINA1*E342K)#Slcw x Homozygous Prkdc Hemizygous Il2rgHomozygous Tg(SERPINA1*E342K)#Slc
Citation
When using the NSG-PiZ mouse strain in a publication, please cite the originating article(s) and include JAX stock #028842 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

RB05 (Maximum)

Pricing & Availability

Available Now

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International

Domestic Pricing for

Commercial & For-Profit

Not-For-Profit & Academic

International Pricing for

Commercial & For-Profit

Not-For-Profit & Academic

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

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Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.

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.

Terms Of Use

General Terms and Conditions

Questions about Terms of Use

Additional Use Restrictions Apply

Research institutions require an MTA, companies require a (no fee) commercial use agreement prior to shipping.

Licensing Information

Phone: 207-288-6470

Email: TechTran@jax.org

Also Known As:NSG-PiZ

Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Expression Data

Control Suggestions

Approximate Controls

Additional Information

Selected References

Prkdcscid

Allele Symbol: Prkdcscid

Il2rgtm1Wjl

Allele Symbol: Il2rgtm1Wjl

Tg(SERPINA1*E342K)#Slcw

Allele Symbol: Tg(SERPINA1*E342K)#Slcw

Disease Terms

Research Areas By Phenotype

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

Genotype: Prkdcscid related

Genotype: Il2rgtm1Wjl related

Mammalian Phenotype Terms by Genotype

References

Additional - Il2rgtm1Wjl related

Additional - Prkdcscid related

Additional - Tg(SERPINA1*E342K)#Slcw related

Genotyping Protocols

Breeding Considerations

Mating System

Citation

Animal Health Reports

Live Mouse

Cryorecovery - Pricing

Payment Terms and Conditions

The Jackson Laboratory's Genotype Promise

Terms Of Use

Additional Use Restrictions Apply

Licensing Information

Prkdc^scid

Allele Symbol: Prkdc^scid

Il2rg^tm1Wjl

Allele Symbol: Il2rg^tm1Wjl

Genotype: Prkdc^scid related

Genotype: Il2rg^tm1Wjl related

Additional - Il2rg^tm1Wjl related

Additional - Prkdc^scid related