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

Status

Available to order

EMMA IDEM:00181
Citation informationRRID:IMSR_EM:00181 

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 nameB6;129S7-Prnptm1Cwe Tg(Prnp)a20Cwe/CweCnrm
Alternative nameTga20
Strain typeTargeted Mutant Strains : Knock-out
Allele/Transgene symbolPrnptm1Cwe, Tg(Prnp)a20Cwe
Gene/Transgene symbolPrnp, Tg(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
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

  • 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
  • Involvement of Cellular Prion Protein in α-Synuclein Transport in Neurons.;Urrea Laura, Segura-Feliu Miriam, Masuda-Suzukake Masami, Hervera Arnau, Pedraz Lucas, García Aznar José Manuel, Vila Miquel, Samitier Josep, Torrents Eduard, Ferrer Isidro, Gavín Rosalina, Hagesawa Masato, Del Río José Antonio, ;2018;Molecular neurobiology;55;1847-1860; 28229331
  • A naturally occurring C-terminal fragment of the prion protein (PrP) delays disease and acts as a dominant-negative inhibitor of PrPSc formation.;Westergard Laura, Turnbaugh Jessie A, Harris David A, ;2011;The Journal of biological chemistry;286;44234-44242; 22025612
  • Visualization of prion infection in transgenic mice expressing green fluorescent protein-tagged prion protein.;Barmada Sami J, Harris David A, ;2005;The Journal of neuroscience : the official journal of the Society for Neuroscience;25;5824-32; 15958749
  • Immunological mimicry of PrPC-PrPSc interactions: antibody-induced PrP misfolding.;Li Li, Guest Will, Huang Alan, Plotkin Steven S, Cashman Neil R, ;2009;Protein engineering, design & selection : PEDS;22;523-9; 19602568
  • Neurotoxicity of prion peptides mimicking the central domain of the cellular prion protein.;Vilches Silvia, Vergara Cristina, Nicolás Oriol, Sanclimens Gloria, Merino Sandra, Varón Sonia, Acosta Gerardo A, Albericio Fernando, Royo Miriam, Del Río José A, Gavín Rosalina, ;2013;PloS one;8;e70881; 23940658
  • Analysis of co-isogenic prion protein deficient mice reveals behavioral deficits, learning impairment, and enhanced hippocampal excitability.;Matamoros-Angles A, Hervera A, Soriano J, Martí E, Carulla P, Llorens F, Nuvolone M, Aguzzi A, Ferrer I, Gruart A, Delgado-García J M, Del Río J A, ;2022;BMC biology;20;17; 35027047
  • Tau Exon 10 Inclusion by PrPC through Downregulating GSK3β Activity.;Lidón Laia, Llaó-Hierro Laura, Nuvolone Mario, Aguzzi Adriano, Ávila Jesús, Ferrer Isidro, Del Río José Antonio, Gavín Rosalina, ;2021;International journal of molecular sciences;22;11971; 34065232
  • Involvement of PrP(C) in kainate-induced excitotoxicity in several mouse strains.;Carulla Patricia, Llorens Franc, Matamoros-Angles Andreu, Aguilar-Calvo Patricia, Espinosa Juan Carlos, Gavín Rosalina, Ferrer Isidre, Legname Giuseppe, Torres Juan Maria, del Río José A, ;2015;Scientific reports;5;62; 26155834
  • The N-terminus of the prion protein is a toxic effector regulated by the C-terminus.;Wu Bei, McDonald Alex J, Markham Kathleen, Rich Celeste B, McHugh Kyle P, Tatzelt Jörg, Colby David W, Millhauser Glenn L, Harris David A, ;2017;eLife;6;; 28527237
  • Differential modulation of NMDA and AMPA receptors by cellular prion protein and copper ions.;Huang Sun, Chen Lina, Bladen Chris, Stys Peter K, Zamponi Gerald W, ;2018;Molecular brain;11;; 30359282

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

Other EMMA strains

Not found what you were looking for? Search here for other strains available from EMMA.


Search
INFRAFRONTIER® and European Mouse Mutant Archive - EMMA® are registered trademarks at the European Union Intellectual Property Office (EUIPO).