B6;129S7-Prnptm1Cwe Tg(Prnp)a20Cwe/CweCnrm

Status

Available to order

EMMA IDEM:00181
International strain nameB6;129S7-Prnptm1Cwe Tg(Prnp)a20Cwe/CweCnrm
Alternative nameTga20
Strain typeTransgenic Strains
Allele/Transgene symbolTg(Prnp)a20Cwe,
Gene/Transgene symbolTg(Prnp)a20Cwe

Information from provider

ProviderAdriano Aguzzi
Provider affiliationUniv.Hosp.Zurich/Inst.Neuropathology
Phenotypic informationReintroduction of Prnp (PrP) constructs on Prnp-/- background. Introduction of half genomic PrP construct (phgPrP; copy number 60). Homozygous breeding.
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

Information from EMMA

Archiving centreCNR, Consiglio Nazionale delle Ricerche, Monterotondo, Italy
Breeding at archiving centreA 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

MGI phenotypes (allele matching)
  • 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

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

Information on how we integrate external resources can be found here

Order

Availabilities

Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

  • Frozen embryos. Delivered in 4 weeks (after paperwork in place). €1740*
  • Rederivation of mice from frozen stock, delivery time available upon request . €3880*

Due to the dynamic nature of our processes strain availability may change at short notice. The local repository manager will advise you in these circumstances.

* In addition users have to cover all the shipping costs (including the cost for returning dry-shippers, where applicable).

More details on pricing and delivery times

Practical information

Genotyping protocol

Example health report
(Current health report will be provided later)

Material Transfer Agreement (MTA)
For this strain no provider MTA is needed. Distribution is based on the EMMA conditions only.

EMMA conditions
Legally binding conditions for the transfer

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