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
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|
|Allele Type||Targeted (Null/Knockout)|
|Allele Synonym(s)||Prnptm1Cwe; targeted mutation 1, Charles Weissmann|
|Gene Symbol and Name||Prnp, prion protein|
|Gene Synonym(s)||PrPSc; AI325101; AltPrP; prion protein, structural locus; Sinc; CD230; GSS; AA960666; Sinc; Prn-i; Prn-p; PrP27-30; PrP33-35C; PrPC; PRIP; Prn-i; Prn-p; PrP; ASCR; expressed sequence AA960666; p27-30; expressed sequence AI325101; CJD; KURU; prion protein, scrapie incubation time regulation; scrapie incubation period; Prn; PrPc|
|Strain of Origin||129S7/SvEvBrd-Hprt+|
|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.
|Heterozygous for Prnp<tm1Cwe>|
We will fulfill your order by providing at least two carriers for each strain ordered. The total number, sex, and genotypes provided will vary, although typically 8 or more animals are provided. Please check genotypes which will be recovered. While the genotypes of all animals produced will be communicated to you prior to scheduling shipment, the genotypes of animals provided may not reflect the mating scheme and genotypes described in the strain description. Animals are typically ready to ship in 11-14 weeks. If a second recovery is required to produce the minimum number of animals, then delivery time would increase to approximately 25 weeks. If we fail to produce animals of the correct genotype, you will not be charged. We cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.
Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation.
|Frozen Mouse Embryo||$2,595.00 per straw or vial|
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