B6;129S7-Prnp^tm1Cwe Tg(Prnp)a20Cwe/CweCnrm

Status	Available to order
EMMA ID	EM:00181
International strain name	B6;129S7-Prnp^tm1Cwe Tg(Prnp)a20Cwe/CweCnrm
Alternative name	Tga20
Strain type	Targeted Mutant Strains : Knock-out
Allele/Transgene symbol	Prnp^tm1Cwe, Tg(Prnp)a20Cwe
Gene/Transgene symbol	Prnp, 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	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 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)

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 PrP^C 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

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