These prion protein-deficient mice are resistant to prion disease and serve as a PrP-deficient background for mouse models of spongiform encephalopathies and neurologic prion disorders.
Stanley Prusiner, University of California, San Francisco
Genetic Background | Generation |
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Allele Type | Gene Symbol | Gene Name |
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Targeted (Null/Knockout) | Prnp | prion protein |
A neo cassette replaces part of exon 3 of the prion protein (Prnp) gene in this strain, abolishing gene function. Prnp encodes the glycoprotein PrPC which is attached to the external surface of central nervous system (CNS) neurons by a glycosylphosphatidyl inositol (GPI) anchor. Conformational changes in PrPC to an infectious form, PrPSc, either by exogenous introduction or as a result of spontaneous mutation, have been linked to the development of spongiform encephalopathies such as scrapie in sheep and Creutzfeld-Jakob disease or GerstmannStraussler-Scheinker syndrome in man. These PrP0/0 mice are viable and fertile. They exhibit decreased copper levels in the brain, impaired synaptic transmission, defective sleep-wake cycles, and altered circadian rhythms. When inoculated with PrPSc, PrP0/0 mice are resistant to prion disease and do not replicate the infectious proteins. These mice serve as a PrPC-deficient background for transgenic mice expressing GPI anchor defects (see (Stock No. 018124).
A targeting vector was designed to replace part of exon 3 of the prion protein (Prnp) gene with a neomycin resistance (neo) cassette. The construct was electroporated into 129S7/SvEvBrd-Hprt+-derived AB1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6J blastocysts and the resulting chimeric males were bred to C57BL/6J females. These Prnp0/0 mice were backcrossed to C57BL/6J mice and subsequently to FVB/NCrl mice for 10 generations. Zygotes from these mice were microinjected with a transgene expressing PRNP lacking the GPI anchor signal (Tg(Prnp-tTA)F959Sbp). Upon arrival at The Jackson Laboratory, Tg959/Prnp0/0 mice were bred to FVB/NJ (Stock No. 001800) for at least one generation. The Tg(Prnp-tTA)F959Sbp transgene was bred out and discarded. A colony of mice containing only the Prnp0/0 allele was established.
Of note, another mouse line containing the Tg(Prnp-tTA)F959Sbp allele was imported and is maintained as Stock No. 018124.
Allele Name | targeted mutation 1, Charles Weissmann |
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Allele Type | Targeted (Null/Knockout) |
Allele Synonym(s) | Prnp trn1Zrch; Prnp-; Prnp0; Prn-p0; Prnp0 Zurich I; Prnptm1ZH1; Prnptm1Zrch; PrnpZH1; PrP0; PrPC Zrch-1; PrPC-KO; Zrch 1 Prnp0; Zrch Prnp0; ZrchI Prnp-; Zurich line |
Gene Symbol and Name | Prnp, prion protein |
Gene Synonym(s) | |
Strain of Origin | 129S7/SvEvBrd-Hprt+ |
Chromosome | 2 |
Molecular Note | A 552 bp fragment of the coding region contained within exon 3 was replaced with a 1.1 kb cassette containing the TK promoter followed by the neomycin gene. |
Mutations Made By | Stanley Prusiner, University of California, San Francisco |
When maintaining a live colony, homozygous mice may be bred together.
When using the FVB.129S7(B6)-Prnptm1Cwe/J mouse strain in a publication, please cite the originating article(s) and include JAX stock #018122 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
---|---|---|
Heterozygous for Prnp<tm1Cwe> |
Frozen Mouse Embryo | FVB.129S7(B6)-Prnp<tm1Cwe>/J | $2595.00 |
Frozen Mouse Embryo | FVB.129S7(B6)-Prnp<tm1Cwe>/J | $2595.00 |
Frozen Mouse Embryo | FVB.129S7(B6)-Prnp<tm1Cwe>/J | $3373.50 |
Frozen Mouse Embryo | FVB.129S7(B6)-Prnp<tm1Cwe>/J | $3373.50 |
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