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.
Dr. Leonard D. Shultz, The Jackson Laboratory
Genetic Background | Generation |
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Allele Type | Gene Symbol | Gene Name |
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Spontaneous | Prkdc | protein kinase, DNA activated, catalytic polypeptide |
Allele Type | Gene Symbol | Gene Name |
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Targeted (Null/Knockout) | Il2rg | interleukin 2 receptor, gamma chain |
Allele Type |
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Transgenic (Inserted expressed sequence, Humanized sequence) |
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
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-Prkdcscid Il2rgtm1Wjl/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-Prkdcscid/J (Stock No. 001303) mice with male mice bearing the X-linked B6.129S4-Il2rgtm1Wjl/J allele (Stock No. 003174). The resulting male mice heterozygous for the Prkdcscid allele and hemizygous for the Il2rgtm1Wjl allele were crossed to female NOD.CB17-Prkdcscid/J (Stock No. 001303) mice for eight generations. Heterozygotes were interbred to produce mice homozygous for the Prkdcscid allele and homozygous (females) or hemizygous (males) for the Il2rgtm1Wjl allele.
Expressed Gene | SERPINA1, serpin family A member 1, human |
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Site of Expression |
Allele Name | severe combined immunodeficiency |
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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-Ighb/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*). |
Allele Name | targeted mutation 1, Warren J Leonard |
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Allele Type | Targeted (Null/Knockout) |
Allele Synonym(s) | [KO]gammac; CD132-; gammac-; gc-; Il2rgtm1Wjll; IL2Rgammanull |
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 |
Allele Name | transgene insertion, Savio L C Woo |
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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. |
When maintaining a live colony, females that are homozygous for the Prkdcscid and Il2rgtm1Wjl alleles and the SERPINA1* transgene are bred to males that are homozygous for Prkdcscid and SERPINA1* transgene, and hemizygous for Il2rgtm1Wjl (Il2rg is an X-linked gene).
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.
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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. 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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