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These prion protein-deficient mice are resistant to prion disease and serve as a PrP-deficient background for mouse models of spongiform encephalopathies and neurologic prion disorders.


The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Congenic; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
Generation Definitions
Donating Investigator Stanley Prusiner,   University of California, San Francisco

A neo cassette replaces part of exon 3 of the prion protein (Prnp) gene in this strain, abolishing gene function. Prnp encodes the glycoprotein PrPC which is attached to the external surface of central nervous system (CNS) neurons by a glycosylphosphatidyl inositol (GPI) anchor. Conformational changes in PrPC to an infectious form, PrPSc, either by exogenous introduction or as a result of spontaneous mutation, have been linked to the development of spongiform encephalopathies such as scrapie in sheep and Creutzfeld-Jakob disease or GerstmannStraussler-Scheinker syndrome in man. These PrP0/0 mice are viable and fertile. They exhibit decreased copper levels in the brain, impaired synaptic transmission, defective sleep-wake cycles, and altered circadian rhythms. When inoculated with PrPSc, PrP0/0 mice are resistant to prion disease and do not replicate the infectious proteins. These mice serve as a PrPC-deficient background for transgenic mice expressing GPI anchor defects (see (Stock No. 018124).

A targeting vector was designed to replace part of exon 3 of the prion protein (Prnp) gene with a neomycin resistance (neo) cassette. The construct was electroporated into 129S7/SvEvBrd-Hprt+-derived AB1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6J blastocysts and the resulting chimeric males were bred to C57BL/6J females. These Prnp0/0 mice were backcrossed to C57BL/6J mice and subsequently to FVB/NCrl mice for 10 generations. Zygotes from these mice were microinjected with a transgene expressing PRNP lacking the GPI anchor signal Tg(Prnp-tTA)F959Sbp/J. Upon arrival at The Jackson Laboratory, Tg959/Prnp0/0 mice were bred to FVB/NJ (Stock No. 001800) for at least one generation. The Tg(Prnp-tTA)F959Sbp transgene was bred out of some mice, and a colony of mice containing only the Prnp0/0 allele was established. Mice containing the Tg(Prnp-tTA)F959Sbp allele are maintained as Stock No. 018124.

Control Information

   001800 FVB/NJ (approximate)
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Prnp
012938   129-Prnptm2Edin/J
016925   129;B6-Del(10Grin3b-Tmem259)1Zang Tg(Prnp-C19ORF6,-GFP)6Zhang/J
003960   129S6-Tg(Prnp-GFP/cre)1Blw/J
005866   B6.Cg-Tg(APP695)3Dbo Tg(PSEN1dE9)S9Dbo/Mmjax
006006   B6.Cg-Tg(Prnp-APP)A-2Dbo/J
008596   B6.Cg-Tg(Prnp-Abca1)EHol/J
006005   B6.Cg-Tg(Prnp-App/APPswe)E1-2Dbo/Mmjax
020783   B6.Cg-Tg(Prnp-FUS)17Ljh/J
021459   B6.Cg-Tg(Prnp-FUS*H517Q)29Ljh/J
019728   B6.Cg-Tg(Prnp-FUS*R495X)78Ljh/J
007180   B6.Cg-Tg(Prnp-ITM2B/APP695*40)1Emcg/J
007182   B6.Cg-Tg(Prnp-ITM2B/APP695*42)A12Emcg/J
006823   B6.Cg-Tg(Prnp-SNCA*A53T)23Mkle/J
010700   B6.Cg-Tg(Prnp-TARDBP*A315T)95Balo/J
009337   B6.FVB-Tg(Prnp-RTN3)2Yanr/J
007002   B6;C3-Tg(Prnp-ITM2B/APP695*42)A12Emcg/Mmjax
008169   B6;C3-Tg(Prnp-MAPT*P301S)PS19Vle/J
004479   B6;C3-Tg(Prnp-SNCA*A53T)83Vle/J
018917   B6;SJL-Tg(Prnp-CCS)17Jlel/J
003378   B6C3-Tg(APP695)3Dbo Tg(PSEN1)5Dbo/J
008216   B6CBA(FVB)-Tg(Prnp-TBP*)71-16Xjl/J
008075   B6CBA-Tg(Prnp-TBP*)105Xjl/J
008083   B6CBA-Tg(Prnp-TBP*)13Xjl/J
003741   B6D2-Tg(Prnp-MAPT)43Vle/J
024841   B6N.Cg-Tg(Prnp-MAPT*P301S)PS19Vle/J
017907   B6N.Cg-Tg(Prnp-TARDBP)96Dwc/J
017933   B6N.Cg-Tg(Prnp-TARDBP*Q331K)103Dwc/J
017930   B6N.Cg-Tg(Prnp-TARDBP*Q331K)109Dwc/J
025402   B6SJL-Tg(Prnp-Immt/SOD1)1Gmnf/J
025403   B6SJL-Tg(Prnp-Immt/SOD1*G93A)7Gmnf/J
016201   B6SJL-Tg(Prnp-TARDBP)4Jlel/J
016203   B6SJL-Tg(Prnp-TARDBP*A315T)23Jlel/J
016608   C57BL/6-Tg(Prnp-TARDBP)3cPtrc/J
017604   C57BL/6-Tg(Prnp-TARDBP*M337V)4Ptrc/J
025104   FVB/N-Tg(tetO/Prnp-MAPT*P301L,-luc)Y74Dbo/J
017678   FVB;129-Pink1tm1Aub Tg(Prnp-SNCA*A53T)AAub/J
017744   FVB;129-Tg(Prnp-SNCA*A53T)AAub/J
017916   STOCK Tg(Prnp-FUS)WT3Cshw/J
016144   STOCK Tg(Prnp-TARDBP)4Jlel/J
016143   STOCK Tg(Prnp-TARDBP*A315T)23Jlel/J
008212   STOCK Tg(SMN2)89Ahmb Smn1tm1Msd Tg(Prnp-SMN)92Ahmb/J
View Strains carrying other alleles of Prnp     (41 strains)


Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- No similarity to the expected human disease phenotype was found. One or more human genes are associated with this human disease. The mouse genotype may involve mutations to orthologs of one or more of these genes, but the phenotype did not resemble the disease.
Creutzfeldt-Jakob Disease; CJD
Gerstmann-Straussler Disease; GSD
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Fatal Familial Insomnia; FFI   (PRNP)
Huntington Disease-Like 1; HDL1   (PRNP)
Kuru, Susceptibility to   (PRNP)
Spongiform Encephalopathy with Neuropsychiatric Features   (PRNP)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.


        involves: 129S7/SvEvBrd * C57BL/6
  • behavior/neurological phenotype
  • *normal* behavior/neurological phenotype
    • normal behavior   (MGI Ref ID J:472)
    • no ataxia or Purkinje cell loss observed   (MGI Ref ID J:67593)
  • nervous system phenotype
  • abnormal CNS synaptic transmission
    • GABA(A) receptor-mediated fast inhibition is weaker in hippocampal slices of mutants than in wild-type mice, indicating impaired synaptic inhibition   (MGI Ref ID J:19448)
    • abnormal inhibitory postsynaptic currents
      • inhibitory postsynaptic currents (ipscs) have significantly slower rising phases and are slower   (MGI Ref ID J:19448)
    • abnormal inhibitory postsynaptic potential
      • fast inhibitory postsynaptic potentials (ipsps) appear weaker in hippocampal slices than in wild-type, however excitatory postsynaptic potentials are normal   (MGI Ref ID J:19448)
    • reduced long term potentiation
      • long term potentiation in CA1 of hippocampal slices is weaker in both the absence and presence of bicuculline   (MGI Ref ID J:19448)
  • decreased brain copper level
    • after 12 months, copper levels in the brain fail to increase with age unlike in wild-type mice   (MGI Ref ID J:126728)
    • copper levels in the synaptosomes are decreased compared to in wild-type mice   (MGI Ref ID J:126728)
  • homeostasis/metabolism phenotype
  • decreased brain copper level
    • after 12 months, copper levels in the brain fail to increase with age unlike in wild-type mice   (MGI Ref ID J:126728)
    • copper levels in the synaptosomes are decreased compared to in wild-type mice   (MGI Ref ID J:126728)
View Research Applications

Research Applications
This mouse can be used to support research in many areas including:

Cell Biology Research
Protein Processing

Genes & Alleles

Gene & Allele Information provided by MGI

Allele Symbol Prnptm1Cwe
Allele Name targeted mutation 1, Charles Weissmann
Allele Type Targeted (Null/Knockout)
Common Name(s) PrP0; PrPC Zrch-1; PrPC-KO; Prn-p0; Prnp trn1Zrch; Prnp-; Prnp0; Prnp0 Zurich I; Prnptm1Zrch; PrnpZH1; Zrch 1 Prnp0; Zrch Prnp0; ZrchI Prnp-; Zurich line;
Mutation Made By Stanley Prusiner,   University of California, San Francisco
Strain of Origin129S7/SvEvBrd-Hprt<+>
Gene Symbol and Name Prnp, prion protein
Chromosome 2
Gene Common Name(s) AA960666; AI325101; ASCR; AltPrP; CD230; CJD; GSS; KURU; PRIP; PrP; PrP27-30; PrP33-35C; PrPC; PrPSc; Prn; Prn-i; Prn-p; Sinc; expressed sequence AA960666; expressed sequence AI325101; p27-30; prion protein, scrapie incubation time regulation; prion protein, structural locus; scrapie incubation period;
Molecular Note A 552 bp fragment of the coding region contained within exon 3 was replaced with a 1.1 kb cassette containing the TK promoter followed by the neomycin gene. [MGI Ref ID J:472] [MGI Ref ID J:83401]


Genotyping Information

Genotyping Protocols

Prnptm1Cwe, Separated MCA
Prnptm1Cwe, Separated PCR

Helpful Links

Genotyping resources and troubleshooting


References provided by MGI

Selected Reference(s)

Bueler H; Fischer M; Lang Y; Bluethmann H; Lipp HP; DeArmond SJ; Prusiner SB; Aguet M; Weissmann C. 1992. Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein [see comments] Nature 356(6370):577-82. [PubMed: 1373228]  [MGI Ref ID J:472]

Additional References

Prnptm1Cwe related

Albanese V; Lawson VA; Hill AF; Cappai R; Di Guardo G; Staikopoulos V; Thacker M; Furness JB; Chiocchetti R. 2008. Evidence for prion protein expression in enteroglial cells of the myenteric plexus of mouse intestine. Auton Neurosci 140(1-2):17-23. [PubMed: 18358791]  [MGI Ref ID J:140002]

Altmeppen HC; Prox J; Krasemann S; Puig B; Kruszewski K; Dohler F; Bernreuther C; Hoxha A; Linsenmeier L; Sikorska B; Liberski PP; Bartsch U; Saftig P; Glatzel M. 2015. The sheddase ADAM10 is a potent modulator of prion disease. Elife 4:. [PubMed: 25654651]  [MGI Ref ID J:219996]

Alvarez-Laviada A; Kadurin I; Senatore A; Chiesa R; Dolphin AC. 2014. The inhibition of functional expression of calcium channels by prion protein demonstrates competition with alpha2delta for GPI-anchoring pathways. Biochem J 458(2):365-74. [PubMed: 24329154]  [MGI Ref ID J:210494]

Anaya ZE; Savistchenko J; Massonneau V; Lacroux C; Andreoletti O; Vilette D. 2011. Recovery of small infectious PrP(res) aggregates from prion-infected cultured cells. J Biol Chem 286(10):8141-8. [PubMed: 21212268]  [MGI Ref ID J:170530]

Arantes C; Nomizo R; Lopes MH; Hajj GN; Lima FR; Martins VR. 2009. Prion protein and its ligand stress inducible protein 1 regulate astrocyte development. Glia 57(13):1439-49. [PubMed: 19243016]  [MGI Ref ID J:156202]

Arsac JN; Betemps D; Morignat E; Feraudet C; Bencsik A; Aubert D; Grassi J; Baron T. 2009. Transmissibility of atypical scrapie in ovine transgenic mice: major effects of host prion protein expression and donor prion genotype. PLoS One 4(10):e7300. [PubMed: 19806224]  [MGI Ref ID J:154107]

Asante EA; Li YG; Gowland I; Jefferys JG; Collinge J. 2004. Pathogenic human prion protein rescues PrP null phenotype in transgenic mice. Neurosci Lett 360(1-2):33-6. [PubMed: 15082172]  [MGI Ref ID J:89280]

Asante EA; Linehan JM; Desbruslais M; Joiner S; Gowland I; Wood AL; Welch J; Hill AF; Lloyd SE; Wadsworth JD; Collinge J. 2002. BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J 21(23):6358-66. [PubMed: 12456643]  [MGI Ref ID J:131494]

Asante EA; Linehan JM; Smidak M; Tomlinson A; Grimshaw A; Jeelani A; Jakubcova T; Hamdan S; Powell C; Brandner S; Wadsworth JD; Collinge J. 2013. Inherited prion disease A117V is not simply a proteinopathy but produces prions transmissible to transgenic mice expressing homologous prion protein. PLoS Pathog 9(9):e1003643. [PubMed: 24086135]  [MGI Ref ID J:214875]

Balducci C; Beeg M; Stravalaci M; Bastone A; Sclip A; Biasini E; Tapella L; Colombo L; Manzoni C; Borsello T; Chiesa R; Gobbi M; Salmona M; Forloni G. 2010. Synthetic amyloid-beta oligomers impair long-term memory independently of cellular prion protein. Proc Natl Acad Sci U S A 107(5):2295-300. [PubMed: 20133875]  [MGI Ref ID J:157527]

Barmada S; Piccardo P; Yamaguchi K; Ghetti B; Harris DA. 2004. GFP-tagged prion protein is correctly localized and functionally active in the brains of transgenic mice. Neurobiol Dis 16(3):527-37. [PubMed: 15262264]  [MGI Ref ID J:91817]

Baumann F; Pahnke J; Radovanovic I; Rulicke T; Bremer J; Tolnay M; Aguzzi A. 2009. Functionally relevant domains of the prion protein identified in vivo. PLoS One 4(9):e6707. [PubMed: 19738901]  [MGI Ref ID J:153629]

Baumann F; Tolnay M; Brabeck C; Pahnke J; Kloz U; Niemann HH; Heikenwalder M; Rulicke T; Burkle A; Aguzzi A. 2007. Lethal recessive myelin toxicity of prion protein lacking its central domain. EMBO J 26(2):538-47. [PubMed: 17245436]  [MGI Ref ID J:117485]

Benvegnu S; Gasperini L; Legname G. 2011. Aged PrP null mice show defective processing of neuregulins in the peripheral nervous system. Mol Cell Neurosci 47(1):28-35. [PubMed: 21334441]  [MGI Ref ID J:177965]

Benvegnu S; Poggiolini I; Legname G. 2010. Neurodevelopmental expression and localization of the cellular prion protein in the central nervous system of the mouse. J Comp Neurol 518(11):1879-91. [PubMed: 20394048]  [MGI Ref ID J:174473]

Benvegnu S; Roncaglia P; Agostini F; Casalone C; Corona C; Gustincich S; Legname G. 2011. Developmental influence of the cellular prion protein on the gene expression profile in mouse hippocampus. Physiol Genomics 43(12):711-25. [PubMed: 21406608]  [MGI Ref ID J:174633]

Beraldo FH; Soares IN; Goncalves DF; Fan J; Thomas AA; Santos TG; Mohammad AH; Roffe M; Calder MD; Nikolova S; Hajj GN; Guimaraes AL; Massensini AR; Welch I; Betts DH; Gros R; Drangova M; Watson AJ; Bartha R; Prado VF; Martins VR; Prado MA. 2013. Stress-inducible phosphoprotein 1 has unique cochaperone activity during development and regulates cellular response to ischemia via the prion protein. FASEB J 27(9):3594-607. [PubMed: 23729591]  [MGI Ref ID J:201097]

Bertuchi FR; Bourgeon DM; Landemberger MC; Martins VR; Cerchiaro G. 2012. PrPC displays an essential protective role from oxidative stress in an astrocyte cell line derived from PrPC knockout mice. Biochem Biophys Res Commun 418(1):27-32. [PubMed: 22222374]  [MGI Ref ID J:181227]

Biasini E; Turnbaugh JA; Massignan T; Veglianese P; Forloni G; Bonetto V; Chiesa R; Harris DA. 2012. The toxicity of a mutant prion protein is cell-autonomous, and can be suppressed by wild-type prion protein on adjacent cells. PLoS One 7(3):e33472. [PubMed: 22428057]  [MGI Ref ID J:187051]

Boudinot E; Tremblay P; Champagnat J; Foutz AS. 2002. Respiratory function in mice lacking or overexpressing the prion protein. Neurosci Lett 323(2):89-92. [PubMed: 11950500]  [MGI Ref ID J:107946]

Boy J; Schmidt T; Wolburg H; Mack A; Nuber S; Bottcher M; Schmitt I; Holzmann C; Zimmermann F; Servadio A; Riess O. 2009. Reversibility of symptoms in a conditional mouse model of spinocerebellar ataxia type 3. Hum Mol Genet 18(22):4282-95. [PubMed: 19666958]  [MGI Ref ID J:154079]

Brandner S; Isenmann S; Raeber A; Fischer M; Sailer A; Kobayashi Y; Marino S; Weissmann C; Aguzzi A. 1996. Normal host prion protein necessary for scrapie-induced neurotoxicity. Nature 379(6563):339-43. [PubMed: 8552188]  [MGI Ref ID J:31134]

Brandner S; Raeber A; Sailer A; Blattler T; Fischer M; Weissmann C; Aguzzi A. 1996. Normal host prion protein (PrPC) is required for scrapie spread within the central nervous system. Proc Natl Acad Sci U S A 93(23):13148-51. [PubMed: 8917559]  [MGI Ref ID J:36560]

Bremer J; Baumann F; Tiberi C; Wessig C; Fischer H; Schwarz P; Steele AD; Toyka KV; Nave KA; Weis J; Aguzzi A. 2010. Axonal prion protein is required for peripheral myelin maintenance. Nat Neurosci 13(3):310-8. [PubMed: 20098419]  [MGI Ref ID J:158343]

Bribian A; Fontana X; Llorens F; Gavin R; Reina M; Garcia-Verdugo JM; Torres JM; de Castro F; del Rio JA. 2012. Role of the cellular prion protein in oligodendrocyte precursor cell proliferation and differentiation in the developing and adult mouse CNS. PLoS One 7(4):e33872. [PubMed: 22529900]  [MGI Ref ID J:187211]

Brown DR. 2003. Prion protein expression modulates neuronal copper content. J Neurochem 87(2):377-85. [PubMed: 14511115]  [MGI Ref ID J:126728]

Brown DR. 1999. Prion protein peptide neurotoxicity can be mediated by astrocytes. J Neurochem 73(3):1105-13. [PubMed: 10461901]  [MGI Ref ID J:56932]

Brown DR; Nicholas RS; Canevari L. 2002. Lack of prion protein expression results in a neuronal phenotype sensitive to stress. J Neurosci Res 67(2):211-24. [PubMed: 11782965]  [MGI Ref ID J:113181]

Brown DR; Schmidt B; Kretzschmar HA. 1998. Effects of copper on survival of prion protein knockout neurons and glia. J Neurochem 70(4):1686-93. [PubMed: 9523587]  [MGI Ref ID J:46891]

Bueler H; Aguzzi A; Sailer A; Greiner RA; Autenried P; Aguet M; Weissmann C. 1993. Mice devoid of PrP are resistant to scrapie. Cell 73(7):1339-47. [PubMed: 8100741]  [MGI Ref ID J:12900]

Bueler H; Raeber A; Sailer A; Fischer M; Aguzzi A; Weissmann C. 1994. High prion and PrPSc levels but delayed onset of disease in scrapie-inoculated mice heterozygous for a disrupted PrP gene. Mol Med 1(1):19-30. [PubMed: 8790598]  [MGI Ref ID J:26616]

Campeau JL; Wu G; Bell JR; Rasmussen J; Sim VL. 2013. Early increase and late decrease of purkinje cell dendritic spine density in prion-infected organotypic mouse cerebellar cultures. PLoS One 8(12):e81776. [PubMed: 24312586]  [MGI Ref ID J:209747]

Chadi S; Young R; Le Guillou S; Tilly G; Bitton F; Martin-Magniette ML; Soubigou-Taconnat L; Balzergue S; Vilotte M; Peyre C; Passet B; Beringue V; Renou JP; Le Provost F; Laude H; Vilotte JL. 2010. Brain transcriptional stability upon prion protein-encoding gene invalidation in zygotic or adult mouse. BMC Genomics 11:448. [PubMed: 20649983]  [MGI Ref ID J:162768]

Christensen HM; Dikranian K; Li A; Baysac KC; Walls KC; Olney JW; Roth KA; Harris DA. 2010. A highly toxic cellular prion protein induces a novel, nonapoptotic form of neuronal death. Am J Pathol 176(6):2695-706. [PubMed: 20472884]  [MGI Ref ID J:161315]

Cisse M; Sanchez PE; Kim DH; Ho K; Yu GQ; Mucke L. 2011. Ablation of cellular prion protein does not ameliorate abnormal neural network activity or cognitive dysfunction in the j20 line of human amyloid precursor protein transgenic mice. J Neurosci 31(29):10427-31. [PubMed: 21775587]  [MGI Ref ID J:174512]

Cohen E; Avrahami D; Frid K; Canello T; Levy Lahad E; Zeligson S; Perlberg S; Chapman J; Cohen OS; Kahana E; Lavon I; Gabizon R. 2013. Snord 3A: a molecular marker and modulator of prion disease progression. PLoS One 8(1):e54433. [PubMed: 23349890]  [MGI Ref ID J:195806]

Coitinho AS; Dietrich MO; Hoffmann A; Dall'Igna OP; Souza DO; R Martins V; R Brentani R; Izquierdo I; Lara DR. 2002. Decreased hyperlocomotion induced by MK-801, but not amphetamine and caffeine in mice lacking cellular prion protein (PrP(C)). Brain Res Mol Brain Res 107(2):190-4. [PubMed: 12425947]  [MGI Ref ID J:80440]

Coitinho AS; Roesler R; Martins VR; Brentani RR; Izquierdo I. 2003. Cellular prion protein ablation impairs behavior as a function of age. Neuroreport 14(10):1375-9. [PubMed: 12876477]  [MGI Ref ID J:89766]

Colling SB; Collinge J; Jefferys JG. 1996. Hippocampal slices from prion protein null mice: disrupted Ca(2+)-activated K+ currents. Neurosci Lett 209(1):49-52. [PubMed: 8734907]  [MGI Ref ID J:36183]

Collinge J; Whittington MA; Sidle KC; Smith CJ; Palmer MS; Clarke AR; Jefferys JG. 1994. Prion protein is necessary for normal synaptic function. Nature 370(6487):295-7. [PubMed: 8035877]  [MGI Ref ID J:19448]

Cordier C; Bencsik A; Philippe S; Betemps D; Ronzon F; Calavas D; Crozet C; Baron T. 2006. Transmission and characterization of bovine spongiform encephalopathy sources in two ovine transgenic mouse lines (TgOvPrP4 and TgOvPrP59). J Gen Virol 87(Pt 12):3763-71. [PubMed: 17098996]  [MGI Ref ID J:136473]

Cortes CJ; Qin K; Cook J; Solanki A; Mastrianni JA. 2012. Rapamycin delays disease onset and prevents PrP plaque deposition in a mouse model of Gerstmann-Straussler-Scheinker disease. J Neurosci 32(36):12396-405. [PubMed: 22956830]  [MGI Ref ID J:221701]

Coulpier M; Messiaen S; Boucreaux D; Eloit M. 2006. Axotomy-induced motoneuron death is delayed in mice overexpressing PrPc. Neuroscience 141(4):1827-34. [PubMed: 16843609]  [MGI Ref ID J:113164]

Crecelius AC; Helmstetter D; Strangmann J; Mitteregger G; Frohlich T; Arnold GJ; Kretzschmar HA. 2008. The brain proteome profile is highly conserved between Prnp-/- and Prnp+/+ mice. Neuroreport 19(10):1027-31. [PubMed: 18580573]  [MGI Ref ID J:137870]

Crozet C; Flamant F; Bencsik A; Aubert D; Samarut J; Baron T. 2001. Efficient transmission of two different sheep scrapie isolates in transgenic mice expressing the ovine PrP gene. J Virol 75(11):5328-34. [PubMed: 11333913]  [MGI Ref ID J:69368]

Curtis J; Errington M; Bliss T; Voss K; MacLeod N. 2003. Age-dependent loss of PTP and LTP in the hippocampus of PrP-null mice. Neurobiol Dis 13(1):55-62. [PubMed: 12758067]  [MGI Ref ID J:126706]

Daude N; Wohlgemuth S; Brown R; Pitstick R; Gapeshina H; Yang J; Carlson GA; Westaway D. 2012. Knockout of the prion protein (PrP)-like Sprn gene does not produce embryonic lethality in combination with PrPC-deficiency. Proc Natl Acad Sci U S A 109(23):9035-40. [PubMed: 22619325]  [MGI Ref ID J:184837]

Devanathan V; Jakovcevski I; Santuccione A; Li S; Lee HJ; Peles E; Leshchyns'ka I; Sytnyk V; Schachner M. 2010. Cellular form of prion protein inhibits Reelin-mediated shedding of Caspr from the neuronal cell surface to potentiate Caspr-mediated inhibition of neurite outgrowth. J Neurosci 30(27):9292-305. [PubMed: 20610764]  [MGI Ref ID J:161770]

Diez M; Groth D; DeArmond SJ; Prusiner SB; Hokfelt T. 2007. Changes in neuropeptide expression in mice infected with prions. Neurobiol Aging 28(5):748-65. [PubMed: 16621165]  [MGI Ref ID J:121978]

Dong J; Li A; Yamaguchi N; Sakaguchi S; Harris DA. 2007. Doppel induces degeneration of cerebellar Purkinje cells independently of Bax. Am J Pathol 171(2):599-607. [PubMed: 17569776]  [MGI Ref ID J:142939]

Dossena S; Imeri L; Mangieri M; Garofoli A; Ferrari L; Senatore A; Restelli E; Balducci C; Fiordaliso F; Salio M; Bianchi S; Fioriti L; Morbin M; Pincherle A; Marcon G; Villani F; Carli M; Tagliavini F; Forloni G; Chiesa R. 2008. Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model. Neuron 60(4):598-609. [PubMed: 19038218]  [MGI Ref ID J:142098]

Douet JY; Lacroux C; Corbiere F; Litaise C; Simmons H; Lugan S; Costes P; Cassard H; Weisbecker JL; Schelcher F; Andreoletti O. 2014. PrP expression level and sensitivity to prion infection. J Virol 88(10):5870-2. [PubMed: 24574409]  [MGI Ref ID J:214428]

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Zabel M; Greenwood C; Thackray AM; Pulford B; Rens W; Bujdoso R. 2009. Perturbation of T-cell development by insertional mutation of a PrP transgene. Immunology 127(2):226-36. [PubMed: 19143847]  [MGI Ref ID J:155660]

Zabel MD; Heikenwalder M; Prinz M; Arrighi I; Schwarz P; Kranich J; von Teichman A; Haas KM; Zeller N; Tedder TF; Weis JH; Aguzzi A. 2007. Stromal complement receptor CD21/35 facilitates lymphoid prion colonization and pathogenesis. J Immunol 179(9):6144-52. [PubMed: 17947689]  [MGI Ref ID J:152995]

Zhang Y; Kim SO; Opsahl-Vital S; Ho SP; Souron JB; Kim C; Giles K; Den Besten PK. 2011. Multiple effects of the cellular prion protein on tooth development. Int J Dev Biol 55(10-12):953-60. [PubMed: 22252492]  [MGI Ref ID J:181928]

de Almeida CJ; Chiarini LB; da Silva JP; E Silva PM; Martins MA; Linden R. 2005. The cellular prion protein modulates phagocytosis and inflammatory response. J Leukoc Biol 77(2):238-46. [PubMed: 15539455]  [MGI Ref ID J:136942]

Health & husbandry

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, homozygous mice may be bred together.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls

Pricing for USA, Canada and Mexico shipping destinations View International Pricing


Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2625.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing


Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3412.50
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Control Information

   001800 FVB/NJ (approximate)
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.

See Terms of Use tab for General Terms and Conditions

The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
Ordering Information
JAX® Mice
Surgical and Preconditioning Services
JAX® Services
Customer Services and Support
Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
Technical Support Email Form

Terms of Use

Terms of Use

General Terms and Conditions

For Licensing and Use Restrictions view the link(s) below:
- Use of MICE by companies or for-profit entities requires a license prior to shipping.

Contact information

General inquiries regarding Terms of Use

Contracts Administration


JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty


In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.