MGI phenotypes (allele matching)
STOCK Prnptm2Edin Tg(Prnp-PRNP)340Jmto/Cnbc
| Status | Available to order |
| EMMA ID | EM:09644 |
| International strain name | STOCK Prnptm2Edin Tg(Prnp-PRNP)340Jmto/Cnbc |
| Alternative name | Prpn tm2Edin tg(moPrpn 129Met-HuPrP-340) Jmtorres |
| Strain type | Transgenic Strains |
| Allele/Transgene symbol | Tg(Prnp-PRNP)340Jmto, |
| Gene/Transgene symbol | Tg(Prnp-PRNP)340Jmto |
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 129Met-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 129Met-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 129Met-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 |
| 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
Literature references
- Sheep and goat BSE propagate more efficiently than cattle BSE in human PrP transgenic mice.;Padilla Danielle, Béringue Vincent, Espinosa Juan Carlos, Andreoletti Olivier, Jaumain Emilie, Reine Fabienne, Herzog Laetitia, Gutierrez-Adan Alfonso, Pintado Belen, Laude Hubert, Torres Juan Maria, ;2011;PLoS pathogens;7;e1001319; 21445238
- 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