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Transgenic Mice Modelling

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Abstract

Although the prion protein (PrP) was discovered in the early 1980s, there is still a considerable lack of knowledge of the normal function of the PrP protein and its precise role in the infectious process of transmissible spongiform encephalopathies (TSEs) or prion diseases. The production and use of a multitude of transgenic mice expressing different forms of PrP has enabled us to increase our knowledge of PrP in health and disease. Using mice expressing PrP from different species, we are able to define the strain of TSE agent infecting a wide range of hosts and model the transmission potential of each agent within and between species. Transgenic mouse models are also utilised in investigating the normal function of PrP, the impact of differential glycosylation in PrP biology and the genetics underlying disease susceptibility. Advances in transgenic technologies have enabled us to control both spatial and temporal expression of PrP, allowing us to define the mechanisms and routes of disease pathogenesis. Transgenic mice also play a vital role in understanding the mechanisms of neurodegeneration in the TSEs, which may also lead to a better understanding of the other protein misfolding diseases such as Alzheimer’s disease.

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Notes

  1. 1.

    Gene targeting is a technique that uses homologous recombination to alter an endogenous gene

  2. 2.

    Sporadic CJD is subclassified via the codon 129 genotype of the host and typed by biochemical properties

References

  • 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):6358–6366

    Article  PubMed  CAS  Google Scholar 

  • Baker CA, Martin D, Manuelidis L (2002) Microglia from Creutzfeldt-Jakob disease-infected brains are infectious and show specific mRNA activation profiles. J Virol 76(21):10905–10913. doi:10.1128/jvi.76.21.10905-10913.2002

    Article  PubMed  CAS  Google Scholar 

  • Barron RM, Thomson V, Jamieson E, Melton DW, Ironside J, Will R, Manson JC (2001) Changing a single amino acid in the N-terminus of murine PrP alters TSE incubation time across three species barriers. EMBO J 20(18):5070–5078

    Article  PubMed  CAS  Google Scholar 

  • Beringue V, Herzog L, Reine F, Le Dur A, Casalone C, Vilotte JL, Laude H (2008) Transmission of atypical bovine prions to mice transgenic for human prion protein. Emerg Infect Dis 14(12):1898–1901

    Article  PubMed  CAS  Google Scholar 

  • Bishop MT, Hart P, Aitchison L, Baybutt HN, Plinston C, Thomson V, Tuzi NL, Head MW, Ironside JW, Will RG, Manson JC (2006) Predicting susceptibility and incubation time of human-to-human transmission of vCJD. Lancet Neurol 5(5):393–398

    Article  PubMed  CAS  Google Scholar 

  • Bishop MT, Ritchie DL, Will RG, Ironside JW, Head MW, Thomson V, Bruce M, Manson JC (2008) No major change in vCJD agent strain after secondary transmission via blood transfusion. PLoS One 3(8):e2878. doi:10.1371/journal.pone.0002878

    Article  PubMed  Google Scholar 

  • Bishop MT, Will RG, Manson JC (2010) Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc Natl Acad Sci U S A 107(26):12005–12010. doi:10.1073/pnas.1004688107 [pii]

    Article  PubMed  CAS  Google Scholar 

  • Blattler T, Brandner S, Raeber AJ, Klein MA, Voigtlander T, Weissmann C, Aguzzi A (1997) PrP-expressing tissue required for transfer of scrapie infectivity from spleen to brain. Nature 389(6646):69–73

    Article  PubMed  CAS  Google Scholar 

  • Bradford BM, Tuzi NL, Feltri ML, McCorquodale C, Cancellotti E, Manson JC (2009) Dramatic reduction of PrP C level and glycosylation in peripheral nerves following PrP knock-out from Schwann cells does not prevent transmissible spongiform encephalopathy neuroinvasion. J Neurosci 29(49):15445–15454. doi:29/49/15445 [pii] 10.1523/JNEUROSCI.4195-09.2009

    Article  PubMed  CAS  Google Scholar 

  • Brown DR, Herms J, Kretzschmar HA (1994a) Mouse cortical cells lacking cellular PrP survive in culture with a neurotoxic PrP fragment. Neuroreport 5(16):2057–2060

    Article  PubMed  CAS  Google Scholar 

  • Brown P, Gibbs CJ Jr, Rodgers-Johnson P, Asher DM, Sulima MP, Bacote A, Goldfarb LG, Gajdusek DC (1994b) Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol 35(5):513–529. doi:10.1002/ana.410350504

    Article  PubMed  CAS  Google Scholar 

  • Bruce MB, Will RG, Fraser H (1999) Comparison of the biological characteristics of BSE and CJD in mice. In: Iqbal K, Swaab DF, Winblad B, Wisniewski HM (eds) Alzheimer’s disease and related disorders. Wiley, Chichester, pp 553–560

    Google Scholar 

  • Bruce ME, McBride PA, Jeffrey M, Scott JR (1994) PrP in pathology and pathogenesis in scrapie-infected mice. Mol Neurobiol 8(2–3):105–112. doi:10.1007/BF02780660

    Article  PubMed  CAS  Google Scholar 

  • Bruce ME, Will RG, Ironside JW, McConnell I, Drummond D, Suttie A, McCardle L, Chree A, Hope J, Birkett C, Cousens S, Fraser H, Bostock CJ (1997) Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent. Nature 389(6650):498–501

    Article  PubMed  CAS  Google Scholar 

  • 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. Nature 356(6370):577–582. doi:10.1038/356577a0

    Article  PubMed  CAS  Google Scholar 

  • Cali I, Castellani R, Yuan J, Al-Shekhlee A, Cohen ML, Xiao X, Moleres FJ, Parchi P, Zou WQ, Gambetti P (2006) Classification of sporadic Creutzfeldt-Jakob disease revisited. Brain 129(Pt 9):2266–2277

    Article  PubMed  Google Scholar 

  • Cancellotti E, Bradford BM, Tuzi NL, Hickey RD, Brown D, Brown KL, Barron RM, Kisielewski D, Piccardo P, Manson JC (2010) Glycosylation of PrPC determines timing of neuroinvasion and targeting in the brain following transmissible spongiform encephalopathy infection by a peripheral route. J Virol 84(7):3464–3475. doi:10.1128/JVI.02374-09 [pii]

    Article  PubMed  CAS  Google Scholar 

  • Cancellotti E, Wiseman F, Tuzi NL, Baybutt H, Monaghan P, Aitchison L, Simpson J, Manson JC (2005) Altered glycosylated PrP proteins can have different neuronal trafficking in brain but do not acquire scrapie-like properties. J Biol Chem 280(52):42909–42918

    Article  PubMed  CAS  Google Scholar 

  • Chesebro B, Race R, Kercher L (2005) Scrapie pathogenesis in brain and retina: Effects of prion protein expression in neurons and astrocytes. J Neurovirol 11(5):476–480. doi:doi:10.1080/13550280500187583

    Article  PubMed  CAS  Google Scholar 

  • Collinge J, Palmer MS, Dryden AJ (1991) Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease. Lancet 337(8755):1441–1442. doi:0140-6736(91)93128-V [pii]

    Article  PubMed  CAS  Google Scholar 

  • Cronier S, Laude H, Peyrin J-M (2004) Prions can infect primary cultured neurons and astrocytes and promote neuronal cell death. Proc Natl Acad Sci U S A 101(33):12271–12276. doi:10.1073/pnas.0402725101

    Article  PubMed  CAS  Google Scholar 

  • Dickinson AG, Meikle VMH, Fraser H (1968) Identification of a gene which controls the incubation period of some strains of scrapie agent in mice. J Comp Pathol 78(3):293–299

    Article  PubMed  CAS  Google Scholar 

  • Eloit M, Adjou K, Coulpier M, Fontaine JJ, Hamel R, Lilin T, Messiaen S, Andreoletti O, Baron T, Bencsik A, Gaelle Biacabe A, Beringue V, Laude H, Le Dur A, Vilotte JL, Comoy E, Deslys JP, Grassi J, Simon S, Lantier F, Sarradin P (2005) BSE agent signatures in a goat. Veterinary Record 156(16):523–524. doi:10.1136/vr.156.16.523-b

    PubMed  Google Scholar 

  • Follet J, Lemaire-Vieille C, Blanquet-Grossard F, Podevin-Dimster V, Lehmann S, Chauvin JP, Decavel JP, Varea R, Grassi J, Fontes M, Cesbron JY (2002) PrP expression and replication by Schwann cells: implications in prion spreading. J Virol 76(5):2434–2439

    Article  PubMed  CAS  Google Scholar 

  • Fraser H, Bruce ME, Chree A, McConnell I, Wells GA (1992) Transmission of bovine spongiform encephalopathy and scrapie to mice. J Gen Virol 73(Pt 8):1891–1897

    Article  PubMed  Google Scholar 

  • Hill AF, Desbruslais M, Joiner S, Sidle KC, Gowland I, Collinge J, Doey LJ, Lantos P (1997) The same prion strain causes vCJD and BSE. Nature 389(6650):448–450. doi:10.1038/38925

    Article  PubMed  CAS  Google Scholar 

  • Hill AF, Joiner S, Wadsworth JD, Sidle KC, Bell JE, Budka H, Ironside JW, Collinge J (2003) Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain 126(Pt 6):1333–1346

    Article  PubMed  Google Scholar 

  • Ilieva H, Polymenidou M, Cleveland DW (2009) Non-cell autonomous toxicity in neurodegenerative disorders: ALS and beyond. J Cell Biol 187(6):761–772. doi:10.1083/jcb.200908164

    Article  PubMed  CAS  Google Scholar 

  • Jeffrey M, Goodsir CM, Bruce ME, McBride PA, Scott JR (1994) Infection-specific prion protein (PrP) accumulates on neuronal plasmalemma in scrapie-infected mice. Ann N Y Acad Sci 724(1):327–330. doi:10.1111/j.1749-6632.1994.tb38923.x

    Article  PubMed  CAS  Google Scholar 

  • Jeffrey M, Goodsir CM, Race RE, Chesebro B (2004) Scrapie-specific neuronal lesions are independent of neuronal PrP expression. Ann Neurol 55(6):781–792. doi:10.1002/ana.20093

    Article  PubMed  CAS  Google Scholar 

  • Jeffrey M, Martin S, Gonzalez L, Foster J, Langeveld JP, van Zijderveld FG, Grassi J, Hunter N (2006) Immunohistochemical features of PrP(d) accumulation in natural and experimental goat transmissible spongiform encephalopathies. J Comp Pathol 134(2–3):171–181. doi:S0021-9975(05)00121-0 [pii] 10.1016/j.jcpa.2005.10.003

    Article  PubMed  CAS  Google Scholar 

  • Kercher L, Favara C, Chan CC, Race R, Chesebro B (2004) Differences in scrapie-induced pathology of the retina and brain in transgenic mice that express hamster prion protein in neurons, astrocytes, or multiple cell types. Am J Pathol 165(6):2055–2067. doi:10.1016/S0002-9440(10)63256-7 [pii]

    Article  PubMed  CAS  Google Scholar 

  • Kercher L, Favara C, Striebel JF, LaCasse R, Chesebro B (2007) Prion protein expression differences in microglia and astroglia influence scrapie-induced neurodegeneration in the retina and brain of transgenic mice. J Virol 81(19):10340–10351. doi:10.1128/JVI.00865-07 [pii]

    Article  PubMed  CAS  Google Scholar 

  • Kimberlin RH, Cole S, Walker CA (1987) Temporary and permanent modifications to a single strain of mouse scrapie on transmission to rats and hamsters. J Gen Virol 68(7):1875–1881. doi:10.1099/0022-1317-68-7-1875

    Article  PubMed  Google Scholar 

  • Kimberlin RH, Walker CA (1979) Pathogenesis of scrapie: agent multiplication in brain at the first and second passage of hamster scrapie in mice. J Gen Virol 42(1):107–117. doi:10.1099/0022-1317-42-1-107

    Article  PubMed  CAS  Google Scholar 

  • Kingsbury D, Kasper K, Stites D, Watson J, Hogan R, Prusiner S (1983) Genetic control of scrapie and Creutzfeldt-Jakob disease in mice. J Immunol 131(1):491–496

    PubMed  CAS  Google Scholar 

  • Kretzschmar HA, Prusiner SB, Stowring LE, DeArmond SJ (1986) Scrapie prion proteins are synthesized in neurons. Am J Pathol 122(1):1–5

    PubMed  CAS  Google Scholar 

  • Llewelyn CA, Hewitt PE, Knight RSG, Amar K, Cousens S, Mackenzie J, Will RG (2004) Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet 363(9407):417–421

    Article  PubMed  CAS  Google Scholar 

  • Lloyd SE, Onwuazor ON, Beck JA, Mallinson G, Farrall M, Targonski P, Collinge J, Fisher EMC (2001) Identification of multiple quantitative trait loci linked to prion disease incubation period in mice. Proc Natl Acad Sci 98(11):6279–6283. doi:10.1073/pnas.101130398

    Article  PubMed  CAS  Google Scholar 

  • Lloyd SE, Thompson SR, Beck JA, Linehan JM, Wadsworth JD, Brandner S, Collinge J, Fisher EM (2004) Identification and characterization of a novel mouse prion gene allele. Mamm Genome 15(5):383–389. doi:10.1007/s00335-004-3041-5

    Article  PubMed  CAS  Google Scholar 

  • Mallucci G, Dickinson A, Linehan J, Klohn PC, Brandner S, Collinge J (2003) Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis. Science 302(5646):871–874. doi:10.1126/science.1090187 302/5646/871 [pii]

    Article  PubMed  CAS  Google Scholar 

  • Mallucci GR, White MD, Farmer M, Dickinson A, Khatun H, Powell AD, Brandner S, Jefferys JG, Collinge J (2007) Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice. Neuron 53(3):325–335. doi:S0896-6273(07)00008-6 [pii] 10.1016/j.neuron.2007.01.005

    Article  PubMed  CAS  Google Scholar 

  • Manolakou K, Beaton J, McConnell I, Farquar C, Manson J, Hastie ND, Bruce M, Jackson IJ (2001) Genetic and environmental factors modify bovine spongiform encephalopathy incubation period in mice. Proc Natl Acad Sci U S A 98(13):7402–7407. doi:10.1073/pnas.121172098 [pii]

    Article  PubMed  CAS  Google Scholar 

  • Manson JC, Clarke AR, Hooper ML, Aitchison L, McConnell I, Hope J (1994a) 129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal. Mol Neurobiol 8(2–3):121–127. doi:10.1007/BF02780662

    Article  PubMed  CAS  Google Scholar 

  • Manson JC, Clarke AR, McBride PA, McConnell I, Hope J (1994b) PrP gene dosage determines the timing but not the final intensity or distribution of lesions in scrapie pathology. Neurodegeneration 3(4):331–340

    PubMed  CAS  Google Scholar 

  • Manson JC, Jamieson E, Baybutt H, Tuzi NL, Barron R, McConnell I, Somerville R, Ironside J, Will R, Sy MS, Melton DW, Hope J, Bostock C (1999) A single amino acid alteration (101L) introduced into murine PrP dramatically alters incubation time of transmissible spongiform encephalopathy. EMBO J 18(23):6855–6864

    Article  PubMed  CAS  Google Scholar 

  • McBride PA, Schulz-Schaeffer WJ, Donaldson M, Bruce M, Diringer H, Kretzschmar HA, Beekes M (2001) Early spread of scrapie from the gastrointestinal tract to the central nervous system involves autonomic fibers of the splanchnic and vagus nerves. J Virol 75(19):9320–9327. doi:10.1128/JVI.75.19.9320-9327.2001

    Article  PubMed  CAS  Google Scholar 

  • Moore RC, Hope J, McBride PA, McConnell I, Selfridge J, Melton DW, Manson JC (1998) Mice with gene targetted prion protein alterations show that Prnp, Sinc and Prni are congruent. Nat Genet 18(2):118–125

    Article  PubMed  CAS  Google Scholar 

  • Moser M, Colello RJ, Pott U, Oesch B (1995) Developmental expression of the prion protein gene in glial cells. Neuron 14(3):509–517

    Article  PubMed  CAS  Google Scholar 

  • Muramoto T, Kitamoto T, Tateishi J, Goto I (1993) Accumulation of abnormal prion protein in mice infected with Creutzfeldt-Jakob disease via intraperitoneal route: a sequential study. Am J Pathol 143(5):1470–1479

    PubMed  CAS  Google Scholar 

  • Padilla D, Beringue V, Espinosa JC, Andreoletti O, Jaumain E, Reine F, Herzog L, Gutierrez-Adan A, Pintado B, Laude H, Torres JM (2011) Sheep and goat BSE propagate more efficiently than cattle BSE in human PrP transgenic mice. PLoS Pathog 7(3):e1001319. doi:10.1371/journal.ppat.1001319

    Article  PubMed  CAS  Google Scholar 

  • Palmer MS, Dryden AJ, Hughes JT, Collinge J (1991) Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature 352(6333):340–342. doi:10.1038/352340a0

    Article  PubMed  CAS  Google Scholar 

  • Parchi P, Giese A, Capellari S, Brown P, Schulz-Schaeffer W, Windl O, Zerr I, Budka H, Kopp N, Piccardo P, Poser S, Rojiani A, Streichemberger N, Julien J, Vital C, Ghetti B, Gambetti P, Kretzschmar H (1999) Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 46(2):224–233

    Article  PubMed  CAS  Google Scholar 

  • Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW (2004) Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 364(9433):527–529

    Article  PubMed  Google Scholar 

  • Plinston C, Hart P, Chong A, Hunter N, Foster J, Piccardo P, Manson JC, Barron RM (2011) Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy infection following passage in sheep. J Virol 85(3):1174–1181. doi:10.1128/JVI.01578-10 [pii]

    Article  PubMed  CAS  Google Scholar 

  • Priola SA, Lawson VA (2001) Glycosylation influences cross-species formation of protease-resistant prion protein. EMBO J 20(23):6692–6699. doi:10.1093/emboj/20.23.6692

    Article  PubMed  CAS  Google Scholar 

  • Race RE, Priola SA, Bessen RA, Ernst D, Dockter J, Rall GF, Mucke L, Chesebro B, Oldstone MB (1995) Neuron-specific expression of a hamster prion protein minigene in transgenic mice induces susceptibility to hamster scrapie agent. Neuron 15(5):1183–1191. doi:0896-6273(95)90105-1 [pii]

    Article  PubMed  CAS  Google Scholar 

  • Raeber AJ, Race RE, Brandner S, Priola SA, Sailer A, Bessen RA, Mucke L, Manson J, Aguzzi A, Oldstone MB, Weissmann C, Chesebro B (1997) Astrocyte-specific expression of hamster prion protein (PrP) renders PrP knockout mice susceptible to hamster scrapie. EMBO J 16(20):6057–6065

    Article  PubMed  CAS  Google Scholar 

  • Ritchie DL, Boyle A, McConnell I, Head MW, Ironside JW, Bruce ME (2009) Transmissions of variant Creutzfeldt-Jakob disease from brain and lymphoreticular tissue show uniform and conserved bovine spongiform encephalopathy-related phenotypic properties on primary and secondary passage in wild-type mice. J Gen Virol 90(Pt 12):3075–3082. doi:10.1099/vir.0.013227-0 [pii]

    Article  PubMed  CAS  Google Scholar 

  • Sandberg MK, Al-Doujaily H, Sigurdson CJ, Glatzel M, O’Malley C, Powell C, Asante EA, Linehan JM, Brandner S, Wadsworth JD, Collinge J (2010) Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein. The Journal of General Virology 91(Pt 10):2651–2657. doi:10.1099/vir.0.024380-0 [pii]

    Article  PubMed  CAS  Google Scholar 

  • Scott M, Foster D, Mirenda C, Serban D, Coufal F, Walchli M, Torchia M, Groth D, Carlson G, DeArmond SJ, Westaway D, Prusiner SB (1989) Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. Cell 59(5):847–857. doi:0092-8674(89)90608-9 [pii]

    Article  PubMed  CAS  Google Scholar 

  • Taraboulos A, Serban D, Prusiner SB (1990) Scrapie prion proteins accumulate in the cytoplasm of persistently infected cultured cells. J Cell Biol 110(6):2117–2132. doi:10.1083/jcb.110.6.2117

    Article  PubMed  CAS  Google Scholar 

  • Tuzi NL, Cancellotti E, Baybutt H, Blackford L, Bradford B, Plinston C, Coghill A, Hart P, Piccardo P, Barron RM, Manson JC (2008) Host PrP glycosylation: a major factor determining the outcome of prion infection. PLoS Biol 6(4):e100. doi:07-PLBI-RA-2655 [pii] 10.1371/journal.pbio.0060100

    Article  PubMed  Google Scholar 

  • Tuzi NL, Clarke AR, Bradford B, Aitchison L, Thomson V, Manson JC (2004) Cre-loxP mediated control of PrP to study transmissible spongiform encephalopathy diseases. Genesis 40(1):1–6

    Article  PubMed  CAS  Google Scholar 

  • Uro-Coste E, Cassard H, Simon S, Lugan S, Bilheude JM, Perret-Liaudet A, Ironside JW, Haik S, Basset-Leobon C, Lacroux C, Peoch K, Streichenberger N, Langeveld J, Head MW, Grassi J, Hauw JJ, Schelcher F, Delisle MB, Andreoletti O (2008) Beyond PrP9res) type 1/type 2 dichotomy in Creutzfeldt-Jakob disease. PLoS Pathog 4(3):e1000029. doi:10.1371/journal.ppat.1000029

    Article  Google Scholar 

  • van Keulen LJM, Schreuder BEC, Meloen RH, Poelen-van den Berg M, Mooij-Harkes G, Vromans MEW, Langeveld JPM (1995) Immumohistochemical detection and localization of prion protein in brain tissue of sheep with natural scrapie. Veter Pathol Online 32(3):299–308. doi:10.1177/030098589503200312

    Google Scholar 

  • Weissmann C, Bueler H, Fischer M, Sailer A, Aguzzi A, Aguet M (1994a) PrP-deficient mice are resistant to scrapie. Ann N Y Acad Sci 724:235–240

    Article  PubMed  CAS  Google Scholar 

  • Weissmann C, Bueler H, Fischer M, Sauer A, Aguet M (1994b) Susceptibility to scrapie in mice is dependent on PrPC. Philos Trans R Soc Lond 343(1306):431–433. doi:10.1098/rstb.1994.0040

    Article  CAS  Google Scholar 

  • Westaway D, Goodman PA, Mirenda CA, McKinley MP, Carlson GA, Prusiner SB (1987) Distinct prion proteins in short and long scrapie incubation period mice. Cell 51(4):651–662

    Article  PubMed  CAS  Google Scholar 

  • Westaway D, Mirenda CA, Foster D, Zebarjadian Y, Scott M, Torchia M, Yang SL, Serban H, DeArmond SJ, Ebeling C et al (1991) Paradoxical shortening of scrapie incubation times by expression of prion protein transgenes derived from long incubation period mice. Neuron 7(1):59–68. doi:0896-6273(91)90074-A [pii]

    Article  PubMed  CAS  Google Scholar 

  • Wilson R, Plinston C, Hunter N, Casalone C, Corona C, Tagliavini F, Suardi S, Ruggerone M, Moda F, Graziano S, Sbriccoli M, Cardone F, Pocchiari M, Ingrosso L, Baron T, Richt J, Andreoletti O, Simmons M, Lockey R, Manson JC, Barron RM (2012) Chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein. J Gen Virol 93(7):1624–1629

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. The Roslin Institute and Royal (Dick) School of Veterinary Studies, University of Edinburgh, Easter Bush, Midlothian, UK

    Abigail B. Diack Ph.D., Rona Wilson Ph.D., Enrico Cancellotti Ph.D. & Barry Bradford M.Sc.

  2. National CJD Research and Surveillance, Western General Hospital, Edinburgh, UK

    Matthew Bishop Ph.D.

  3. Division of Neurobiology, The Roslin Institute and Royal (Dick) School of Veterinary Studies, University of Edinburgh, Easter Bush, EH25 9RG, UK, Edinburgh

    Jean C. Manson Ph.D.

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  1. Abigail B. Diack Ph.D.
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  2. Rona Wilson Ph.D.
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  3. Enrico Cancellotti Ph.D.
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  4. Barry Bradford M.Sc.
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  5. Matthew Bishop Ph.D.
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  6. Jean C. Manson Ph.D.
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Correspondence to Jean C. Manson Ph.D. .

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Editors and Affiliations

  1. , Department of Pathology, Case Western Reserve University School o, 2085 Adelbert Road, Cleveland, 44106, Ohio, USA

    Wen-Quan Zou

  2. , Department of Neuropathology, Case Western Reserve University School o, 2085 Adelbert Road, Cleveland, 44106, Ohio, USA

    Pierluigi Gambetti

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Diack, A.B., Wilson, R., Cancellotti, E., Bradford, B., Bishop, M., Manson, J.C. (2013). Transgenic Mice Modelling. In: Zou, WQ., Gambetti, P. (eds) Prions and Diseases. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5338-3_10

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