- reduced long term potentiation / MGI
- abnormal CNS synaptic transmission / MGI
- abnormal inhibitory postsynaptic potential / MGI
- abnormal inhibitory postsynaptic currents / MGI
- behavior/neurological phenotype / MGI
- decreased brain copper level / MGI
- tremors / MGI
- abnormal cerebellum morphology / MGI
- decreased Purkinje cell number / MGI
B6;129S7-Prnptm1Cwe Tg(Prnp)a20Cwe/CweCnrm
Status | Available to order |
EMMA ID | EM:00181 |
International strain name | B6;129S7-Prnptm1Cwe Tg(Prnp)a20Cwe/CweCnrm |
Alternative name | Tga20 |
Strain type | Transgenic Strains |
Allele/Transgene symbol | Tg(Prnp)a20Cwe, |
Gene/Transgene symbol | Tg(Prnp)a20Cwe |
Information from provider
Provider | Adriano Aguzzi |
Provider affiliation | Univ.Hosp.Zurich/Inst.Neuropathology |
Phenotypic information | Reintroduction of Prnp (PrP) constructs on Prnp-/- background. Introduction of half genomic PrP construct (phgPrP; copy number 60). Homozygous breeding. |
References |
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Information from EMMA
Archiving centre | CNR, Consiglio Nazionale delle Ricerche, Monterotondo, Italy |
Breeding at archiving centre | A sub-strain with the same mutation and genetic background is maintained at the repository at the Institut de Transgenose, INTRAGENE, Orléans, France: EM:05304 |
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
- Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie.;Fischer M, Rülicke T, Raeber A, Sailer A, Moser M, Oesch B, Brandner S, Aguzzi A, Weissmann C, ;1996;The EMBO journal;15;1255-64; 8635458
- Transgenic fatal familial insomnia mice indicate prion infectivity-independent mechanisms of pathogenesis and phenotypic expression of disease.;Bouybayoune Ihssane, Mantovani Susanna, Del Gallo Federico, Bertani Ilaria, Restelli Elena, Comerio Liliana, Tapella Laura, Baracchi Francesca, Fernández-Borges Natalia, Mangieri Michela, Bisighini Cinzia, Beznoussenko Galina V, Paladini Alessandra, Balducci Claudia, Micotti Edoardo, Forloni Gianluigi, Castilla Joaquín, Fiordaliso Fabio, Tagliavini Fabrizio, Imeri Luca, Chiesa Roberto, ;2015;PLoS pathogens;11;e1004796; 25880443
- Expression of mutant or cytosolic PrP in transgenic mice and cells is not associated with endoplasmic reticulum stress or proteasome dysfunction.;Quaglio Elena, Restelli Elena, Garofoli Anna, Dossena Sara, De Luigi Ada, Tagliavacca Luigina, Imperiale Daniele, Migheli Antonio, Salmona Mario, Sitia Roberto, Forloni Gianluigi, Chiesa Roberto, ;2011;PloS one;6;e19339; 21559407
- The N-terminal, polybasic region is critical for prion protein neuroprotective activity.;Turnbaugh Jessie A, Westergard Laura, Unterberger Ursula, Biasini Emiliano, Harris David A, ;2011;PloS one;6;e25675; 21980526
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