MGI phenotypes (allele matching)
STOCK Prnptm2Edin Tg(Prnp-PRNP)340Jmto/Cnbc
| Status | Available to order |
| EMMA ID | EM:09644 |
| Citation information | RRID:IMSR_EM:09644 Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information. |
| International strain name | STOCK Prnptm2Edin Tg(Prnp-PRNP)340Jmto/Cnbc |
| Alternative name | Prpn tm2Edin tg(moPrpn 129Met-HuPrP-340) Jmtorres |
| Strain type | Targeted Mutant Strains : Knock-out |
| Allele/Transgene symbol | Prnptm2Edin, Tg(Prnp-PRNP)340Jmto |
| Gene/Transgene symbol | Prnp, 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
MGI phenotypes (gene matching)
- impaired fertilization / MGI
- abnormal spleen morphology / MGI
- tremors / MGI
- abnormal cerebral cortex morphology / MGI
- abnormal hippocampus morphology / MGI
- abnormal olfactory bulb morphology / MGI
- abnormal thalamus morphology / MGI
- abnormal cerebellum morphology / MGI
- Purkinje cell degeneration / MGI
- decreased Purkinje cell number / MGI
- abnormal cerebellar molecular layer / MGI
- thin cerebellar molecular layer / MGI
- abnormal retina morphology / MGI
- ataxia / MGI
- hypoactivity / MGI
- impaired coordination / MGI
- reduced long term potentiation / MGI
- abnormal sleep pattern / MGI
- abnormal body temperature homeostasis / MGI
- male infertility / MGI
- premature death / MGI
- abnormal muscle physiology / MGI
- abnormal brain morphology / MGI
- no abnormal phenotype detected / MGI
- gliosis / MGI
- abnormal CNS synaptic transmission / MGI
- neurodegeneration / MGI
- spongiform encephalopathy / MGI
- decreased vertical activity / MGI
- abnormal inhibitory postsynaptic potential / MGI
- abnormal inhibitory postsynaptic currents / MGI
- no phenotypic analysis / MGI
- increased neuron apoptosis / MGI
- neuron degeneration / MGI
- astrocytosis / MGI
- abnormal voluntary movement / MGI
- nervous system phenotype / MGI
- abnormal nervous system morphology / MGI
- impaired acrosome reaction / MGI
- abnormal behavior / MGI
- abnormal neuronal precursor proliferation / MGI
- decreased susceptibility to prion infection / MGI
- increased susceptibility to prion infection / MGI
- behavior/neurological phenotype / MGI
- immune system phenotype / MGI
- teratozoospermia / MGI
- brain vacuoles / MGI
- abnormal brain white matter morphology / MGI
- abnormal hippocampus CA1 region morphology / MGI
- decreased neuron number / MGI
- abnormal neuron differentiation / MGI
- abnormal neuron proliferation / MGI
- decreased brain copper level / MGI
- enlarged brain ventricles / MGI
- cerebellum atrophy / MGI
- altered susceptibility to prion infection / MGI
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