MGI phenotypes (allele matching)
STOCK Prnptm2Edin Tg(Prnp-PRNP)001Jmto/Cnbc
| Status | Available to order |
| EMMA ID | EM:05416 |
| Citation information | RRID:IMSR_EM:05416 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)001Jmto/Cnbc |
| Alternative name | tg(moPrpn PoPrP)001 Jmto |
| Strain type | Targeted Mutant Strains : Knock-out |
| Allele/Transgene symbol | Prnptm2Edin, Tg(Prnp-PRNP)001Jmto |
| Gene/Transgene symbol | Prnp, Tg(Prnp-PRNP)001Jmto |
Information from provider
| Provider | Juan Maria Torres |
| Provider affiliation | CISA, INIA |
| Genetic information | Reintroduction of porcine PrP constructs under the control of a murine PrP promoter on a murine PrPn -/- background. |
| Phenotypic information | Overexpression of porcine PrP in mice lacking expression of murine PrP. Level of expression of bovine PrP in brain is approximately 4-fold that in pig brain. These mice show a differential susceptibility to several prion diseases. |
| 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 12 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 (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
- Subclinical bovine spongiform encephalopathy infection in transgenic mice expressing porcine prion protein.;Castilla Joaquín, Gutiérrez-Adán Alfonso, Brun Alejandro, Doyle Deirdre, Pintado Belén, Ramírez Miguel A, Salguero Francisco J, Parra Beatriz, Segundo Fayna Díaz San, Sánchez-Vizcaíno José M, Rogers Mark, Torres Juan M, ;2004;The Journal of neuroscience : the official journal of the Society for Neuroscience;24;5063-9; 15163699
- Transgenic mice expressing porcine prion protein resistant to classical scrapie but susceptible to sheep bovine spongiform encephalopathy and atypical scrapie.;Espinosa Juan Carlos, Herva María Eugenia, Andréoletti Olivier, Padilla Danielle, Lacroux Caroline, Cassard Hervé, Lantier Isabelle, Castilla Joaquin, Torres Juan Maria, ;2009;Emerging infectious diseases;15;1214-21; 19751582