STOCK Prnptm2Edin Tg(Prnp-PRNP)110Jmto/Cnbc
| Status | Available to order |
| EMMA ID | EM:05415 |
| Citation information | RRID:IMSR_EM:05415 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)110Jmto/Cnbc |
| Alternative name | Prpn tm2Edin tg(moPrpn BoPrP)110 Jmto |
| Strain type | Targeted Mutant Strains : Knock-out |
| Allele/Transgene symbol | Prnptm2Edin, Tg(Prnp-PRNP)110Jmto |
| Gene/Transgene symbol | Prnp, Tg(Prnp-PRNP)110Jmto |
Information from provider
| Provider | Juan Maria Torres |
| Provider affiliation | CISA, INIA |
| Genetic information | Reintroduction of bovine PrP constructs under the control of a murine PrP promoter on a murine PrPn -/- background. |
| Phenotypic information | Overexpression of bovine PrP in mice lacking expression of murine PrP. Level of expression of bovine PrP in brain is approximately 8-fold that in bovine brain. These mice are very susceptible to bovine prions. |
| Breeding history | The original line was produced on embryos obtained crossing B6CBAF1 females with 129P2/OlaHsd males. Founders were crossed to obtain homozygosis for both mutations without a specific breeding scheme. Mice are since then kept by crossing brother to sister. Approximately 15 generations. |
| References |
|
| 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 males, homozygous mixed females |
| Stage of embryos | 8-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)
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
- Early detection of PrPres in BSE-infected bovine PrP transgenic mice.;Castilla J, Gutiérrez Adán A, Brun A, Pintado B, Ramírez M A, Parra B, Doyle D, Rogers M, Salguero F J, Sánchez C, Sánchez-Vizcaíno J M, Torres J M, ;2003;Archives of virology;148;677-91; 12664293
- Different behavior toward bovine spongiform encephalopathy infection of bovine prion protein transgenic mice with one extra repeat octapeptide insert mutation.;Castilla J, Gutiérrez-Adán A, Brun A, Pintado B, Parra B, Ramírez M A, Salguero F J, Díaz San Segundo F, Rábano A, Cano M J, Torres J M, ;2004;The Journal of neuroscience : the official journal of the Society for Neuroscience;24;2156-64; 14999066
- Vertical transmission of bovine spongiform encephalopathy prions evaluated in a transgenic mouse model.;Castilla J, Brun A, Díaz-San Segundo F, Salguero F J, Gutiérrez-Adán A, Pintado B, Ramírez M A, del Riego L, Torres J M, ;2005;Journal of virology;79;8665-8; 15956610
- Sheep-passaged bovine spongiform encephalopathy agent exhibits altered pathobiological properties in bovine-PrP transgenic mice.;Espinosa Juan Carlos, Andréoletti Olivier, Castilla Joaquín, Herva María Eugenia, Morales Mónica, Alamillo Elia, San-Segundo Fayna Díaz, Lacroux Caroline, Lugan Séverine, Salguero Francisco Javier, Langeveld Jan, Torres Juan María, ;2007;Journal of virology;81;835-43; 17079295
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