STOCK Prnptm2Edin Tg(Prnp-PRNP)361Jmto/Cnbc
Status | Available to order |
EMMA ID | EM:09646 |
International strain name | STOCK Prnptm2Edin Tg(Prnp-PRNP)361Jmto/Cnbc |
Alternative name | Prpn tm2Edin tg(moPrpn 129Val-HuPrP-361) Jmtorres |
Strain type | Transgenic Strains |
Allele/Transgene symbol | Tg(Prnp-PRNP)361Jmto |
Gene/Transgene symbol | Tg(Prnp-PRNP)361Jmto |
Information from provider
Provider | Juan María Torres |
Provider affiliation | Centro de Investigación en Sanidad Animal (CISA-INIA), Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria (INIA) |
Genetic information | Reintroduction of 129Val-Human PrP constructs under the control of a murine PrP promoter on a murine PrPn -/- background |
Phenotypic information | Homozygous:Overexpression of bovine PrP in mice lacking expression of murine PrP. Level expression of 129Val-Human PrP in brain is approximately 2-fold that in human brain. These mice are very susceptible to human prion transmission and adequate models to determine human susceptibility to prions from other species.Heterozygous:Level expression of 129Val-Human PrP in brain of hemizygous mice is approximately 2-fold that in human brain. |
Breeding history | The original line was produced on embryos obtained crossing B6CBAF1 females with 129P2/OlaHsd males. Founders were crossed to obtain homozygosity for both mutations without a specific breeding scheme. Mice are since then kept crossing brother to sister. Approximately 15 generations. |
References |
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Homozygous fertile | yes |
Homozygous viable | yes |
Homozygous matings required | yes |
Immunocompromised | no |
Information from EMMA
Archiving centre | CNB-CSIC, Centro Nacional de Biotecnologia, Madrid, Spain |
Animals used for archiving | homozygous mixed, homozygous mixed |
Stage of embryos | 2-cell |
Disease and phenotype information
Orphanet associated rare diseases, based on orthologous gene matching
- Inherited Creutzfeldt-Jakob disease / Orphanet_282166
- Familial Alzheimer-like prion disease / Orphanet_280397
- Huntington disease-like 1 / Orphanet_157941
- PrP systemic amyloidosis / Orphanet_397606
- Fatal familial insomnia / Orphanet_466
- Gerstmann-Straussler-Scheinker syndrome / Orphanet_356
- Sporadic fatal insomnia / Orphanet_586130
MGI phenotypes (allele matching)
Literature references
- Evidence for zoonotic potential of ovine scrapie prions.;Cassard Hervé, Torres Juan-Maria, Lacroux Caroline, Douet Jean-Yves, Benestad Sylvie L, Lantier Frédéric, Lugan Séverine, Lantier Isabelle, Costes Pierrette, Aron Naima, Reine Fabienne, Herzog Laetitia, Espinosa Juan-Carlos, Beringue Vincent, Andréoletti Olivier, ;2014;Nature communications;5;5821; 25510416
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