B6.129S7-Prnptm1Cwe/Orl

Status

Available to order

EMMA IDEM:01723
Citation informationRRID:IMSR_EM:01723 

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/Orl
Alternative nameprp BL6
Strain typeTargeted Mutant Strains : Knock-out
Allele/Transgene symbolPrnptm1Cwe
Gene/Transgene symbolPrnp

Information from provider

ProviderCh Weissmann, Claude Carnaud
Provider affiliationHopital St Antoine, Paris
Genetic informationA 552 bp sequence of the coding region within exon 3 was replaced with a 1.1 kb cassette containing the TK promoter followed by the neomycin gene.
Phenotypic informationResistant to prion infection.
Breeding history11 backcrosses to C57BL/6.
References
  • Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein.;Büeler H, Fischer M, Lang Y, Bluethmann H, Lipp H P, DeArmond S J, Prusiner S B, Aguet M, Weissmann C, ;1992;Nature;356;577-82; 1373228

Information from EMMA

Archiving centreInstitut de Transgenose, INTRAGENE, Orléans, France
Animals used for archivinghomozygous males, wild-type C57BL/6J females

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

  • Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein.;Büeler H, Fischer M, Lang Y, Bluethmann H, Lipp H P, DeArmond S J, Prusiner S B, Aguet M, Weissmann C, ;1992;Nature;356;577-82; 1373228
  • Mutant PrP suppresses glutamatergic neurotransmission in cerebellar granule neurons by impairing membrane delivery of VGCC α(2)δ-1 Subunit.;Senatore Assunta, Colleoni Simona, Verderio Claudia, Restelli Elena, Morini Raffaella, Condliffe Steven B, Bertani Ilaria, Mantovani Susanna, Canovi Mara, Micotti Edoardo, Forloni Gianluigi, Dolphin Annette C, Matteoli Michela, Gobbi Marco, Chiesa Roberto, ;2012;Neuron;74;300-13; 22542184
  • 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
  • Synthetic amyloid-beta oligomers impair long-term memory independently of cellular prion protein.;Balducci Claudia, Beeg Marten, Stravalaci Matteo, Bastone Antonio, Sclip Alessandra, Biasini Emiliano, Tapella Laura, Colombo Laura, Manzoni Claudia, Borsello Tiziana, Chiesa Roberto, Gobbi Marco, Salmona Mario, Forloni Gianluigi, ;2010;Proceedings of the National Academy of Sciences of the United States of America;107;2295-300; 20133875
  • Inhibition of IL-1β Signaling Normalizes NMDA-Dependent Neurotransmission and Reduces Seizure Susceptibility in a Mouse Model of Creutzfeldt-Jakob Disease.;Bertani Ilaria, Iori Valentina, Trusel Massimo, Maroso Mattia, Foray Claudia, Mantovani Susanna, Tonini Raffaella, Vezzani Annamaria, Chiesa Roberto, ;2017;The Journal of neuroscience : the official journal of the Society for Neuroscience;37;10278-10289; 28924012
  • Sleep inhibition induced by amyloid-β oligomers is mediated by the cellular prion protein.;Del Gallo Federico, Bianchi Susanna, Bertani Ilaria, Messa Massimo, Colombo Laura, Balducci Claudia, Salmona Mario, Imeri Luca, Chiesa Roberto, ;2021;Journal of sleep research;30;e13187; 32902030
  • Mutant prion proteins increase calcium permeability of AMPA receptors, exacerbating excitotoxicity.;Ghirardini Elsa, Restelli Elena, Morini Raffaella, Bertani Ilaria, Ortolan Davide, Perrucci Fabio, Pozzi Davide, Matteoli Michela, Chiesa Roberto, ;2020;PLoS pathogens;16;e1008654; 32673372
  • Cellular prion protein neither binds to alpha-synuclein oligomers nor mediates their detrimental effects.;La Vitola Pietro, Beeg Marten, Balducci Claudia, Santamaria Giulia, Restelli Elena, Colombo Laura, Caldinelli Laura, Pollegioni Loredano, Gobbi Marco, Chiesa Roberto, Forloni Gianluigi, ;2019;Brain : a journal of neurology;142;249-254; 30601948
  • Doxycycline rescues recognition memory and circadian motor rhythmicity but does not prevent terminal disease in fatal familial insomnia mice.;Lavigna Giada, Masone Antonio, Bouybayoune Ihssane, Bertani Ilaria, Lucchetti Jacopo, Gobbi Marco, Porcu Luca, Zordan Stefano, Rigamonti Mara, Imeri Luca, Restelli Elena, Chiesa Roberto, ;2021;Neurobiology of disease;158;105455; 34358614
  • Activation of Src family kinase ameliorates secretory trafficking in mutant prion protein cells.;Restelli Elena, Capone Vanessa, Pozzoli Manuela, Ortolan Davide, Quaglio Elena, Corbelli Alessandro, Fiordaliso Fabio, Beznoussenko Galina V, Artuso Vladimiro, Roiter Ignazio, Sallese Michele, Chiesa Roberto, ;2021;The Journal of biological chemistry;296;100490; 33662396
  • Cell type-specific neuroprotective activity of untranslocated prion protein.;Restelli Elena, Fioriti Luana, Mantovani Susanna, Airaghi Simona, Forloni Gianluigi, Chiesa Roberto, ;2010;PloS one;5;e13725; 21060848
  • Epitope scanning indicates structural differences in brain-derived monomeric and aggregated mutant prion proteins related to genetic prion diseases.;Tapella Laura, Stravalaci Matteo, Bastone Antonio, Biasini Emiliano, Gobbi Marco, Chiesa Roberto, ;2013;The Biochemical journal;454;417-25; 23808898
  • 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
  • The inhibition of functional expression of calcium channels by prion protein demonstrates competition with α2δ for GPI-anchoring pathways.;Alvarez-Laviada Anita, Kadurin Ivan, Senatore Assunta, Chiesa Roberto, Dolphin Annette C, ;2014;The Biochemical journal;458;365-74; 24329154
  • Synaptic dysfunction in prion diseases: a trafficking problem?;Senatore Assunta, Restelli Elena, Chiesa Roberto, ;2013;International journal of cell biology;2013;543803; 24369467
  • Prions activate a p38 MAPK synaptotoxic signaling pathway.;Fang Cheng, Wu Bei, Le Nhat T T, Imberdis Thibaut, Mercer Robert C C, Harris David A, ;2018;PLoS pathogens;14;e1007283; 30235355
  • The N-terminal, polybasic region of PrP(C) dictates the efficiency of prion propagation by binding to PrP(Sc).;Turnbaugh Jessie A, Unterberger Ursula, Saá Paula, Massignan Tania, Fluharty Brian R, Bowman Frederick P, Miller Michael B, Supattapone Surachai, Biasini Emiliano, Harris David A, ;2012;The Journal of neuroscience : the official journal of the Society for Neuroscience;32;8817-30; 22745483
  • Spongiform pathology in mouse CNS lacking 'neuropathy target esterase' and cellular prion protein.;Rosenbluth Jack, Schiff Rolf, Lam Pokman, Nuriel Tal, Chao Moses V, ;2009;Neurobiology of disease;35;433-7; 19524041
  • 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;17; 28527237
  • Tau oligomers impair memory and synaptic plasticity through the cellular prion protein.;Balducci Claudia, Orsini Franca, Cerovic Milica, Beeg Marten, Rocutto Beatrice, Dacomo Letizia, Masone Antonio, Busani Eleonora, Raimondi Ilaria, Lavigna Giada, Chen Po-Tao, Leva Susanna, Colombo Laura, Zucchelli Chiara, Musco Giovanna, Kanaan Nicholas M, Gobbi Marco, Chiesa Roberto, Fioriti Luana, Forloni Gianluigi, ;2025;Acta neuropathologica communications;13;; 39871396

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