- 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 | Targeted Mutant Strains : Knock-out |
Allele/Transgene symbol | Prnptm1Cwe, 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 |
|
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)
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
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