Literature DB >> 10611351

Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans.

M R Scott1, R Will, J Ironside, H O Nguyen, P Tremblay, S J DeArmond, S B Prusiner.   

Abstract

There is growing concern that bovine spongiform encephalopathy (BSE) may have passed from cattle to humans. We report here that transgenic (Tg) mice expressing bovine (Bo) prion protein (PrP) serially propagate BSE prions and that there is no species barrier for transmission from cattle to Tg(BoPrP) mice. These same mice were also highly susceptible to a new variant of Creutzfeldt-Jakob disease (nvCJD) and natural sheep scrapie. The incubation times (approximately 250 days), neuropathology, and disease-causing PrP isoforms in Tg(BoPrP)Prnp(0/0) mice inoculated with nvCJD and BSE brain extracts were indistinguishable and differed dramatically from those seen in these mice injected with natural scrapie prions. Our findings provide the most compelling evidence to date that prions from cattle with BSE have infected humans and caused fatal neurodegeneration.

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Year:  1999        PMID： 10611351      PMCID： PMC24786          DOI： 10.1073/pnas.96.26.15137

Source DB:  PubMed          Journal:  Proc Natl Acad Sci U S A        ISSN： 0027-8424            Impact factor:   11.205


  43 in total

1.  Deaths from variant Creutzfeldt-Jakob disease.

Authors:  R G Will; S N Cousens; C P Farrington; P G Smith; R S Knight; J W Ironside
Journal:  Lancet       Date:  1999-03-20       Impact factor: 79.321

2.  BSE transmission to macaques.

Authors:  C I Lasmézas; J P Deslys; R Demaimay; K T Adjou; F Lamoury; D Dormont; O Robain; J Ironside; J J Hauw
Journal:  Nature       Date:  1996-06-27       Impact factor: 49.962

3.  Prediction of future BSE spread.

Authors:  D J Stekel; M A Nowak; T R Southwood
Journal:  Nature       Date:  1996-05-09       Impact factor: 49.962

4.  New variant of Creutzfeldt-Jakob disease in a 26-year-old French man.

Authors:  G Chazot; E Broussolle; C l Lapras; T Blättler; A Aguzzi; N Kopp
Journal:  Lancet       Date:  1996-04-27       Impact factor: 79.321

5.  Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein.

Authors:  G C Telling; M Scott; J Mastrianni; R Gabizon; M Torchia; F E Cohen; S J DeArmond; S B Prusiner
Journal:  Cell       Date:  1995-10-06       Impact factor: 41.582

6.  Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein.

Authors:  G C Telling; M Scott; K K Hsiao; D Foster; S L Yang; M Torchia; K C Sidle; J Collinge; S J DeArmond; S B Prusiner
Journal:  Proc Natl Acad Sci U S A       Date:  1994-10-11       Impact factor: 11.205

Review 7.  The neuropathology and epidemiology of bovine spongiform encephalopathy.

Authors:  G A Wells; J W Wilesmith
Journal:  Brain Pathol       Date:  1995-01       Impact factor: 6.508

8.  Experimental infection of cattle with the agents of transmissible mink encephalopathy and scrapie.

Authors:  M M Robinson; W J Hadlow; D P Knowles; T P Huff; P A Lacy; R F Marsh; J R Gorham
Journal:  J Comp Pathol       Date:  1995-10       Impact factor: 1.311

9.  A new variant of Creutzfeldt-Jakob disease in the UK.

Authors:  R G Will; J W Ironside; M Zeidler; S N Cousens; K Estibeiro; A Alperovitch; S Poser; M Pocchiari; A Hofman; P G Smith
Journal:  Lancet       Date:  1996-04-06       Impact factor: 79.321

10.  Neuron-specific expression of a hamster prion protein minigene in transgenic mice induces susceptibility to hamster scrapie agent.

Authors:  R E Race; S A Priola; R A Bessen; D Ernst; J Dockter; G F Rall; L Mucke; B Chesebro; M B Oldstone
Journal:  Neuron       Date:  1995-11       Impact factor: 17.173
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  124 in total

1.  Mimicking dominant negative inhibition of prion replication through structure-based drug design.

Authors:  V Perrier; A C Wallace; K Kaneko; J Safar; S B Prusiner; F E Cohen
Journal:  Proc Natl Acad Sci U S A       Date:  2000-05-23       Impact factor: 11.205

Review 2.  New variant Creutzfeldt-Jakob disease: the epidemic that never was.

Authors:  G A Venters
Journal:  BMJ       Date:  2001-10-13

3.  Conformation-dependent high-affinity monoclonal antibodies to prion proteins.

Authors:  Larry H Stanker; Ana V Serban; Elisa Cleveland; Robert Hnasko; Azucena Lemus; Jiri Safar; Stephen J DeArmond; Stanley B Prusiner
Journal:  J Immunol       Date:  2010-06-07       Impact factor: 5.422

Review 4.  Transgenesis applied to transmissible spongiform encephalopathies.

Authors:  Jean-Luc Vilotte; Hubert Laude
Journal:  Transgenic Res       Date:  2002-12       Impact factor: 2.788

5.  Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases.

Authors:  Donald A Collie; David M Summers; Robin J Sellar; James W Ironside; Sarah Cooper; Martin Zeidler; Richard Knight; Robert G Will
Journal:  AJNR Am J Neuroradiol       Date:  2003-09       Impact factor: 3.825

6.  Variant Creutzfeldt-Jakob disease.

Authors:  J W Ironside
Journal:  Vet Res Commun       Date:  2003-09       Impact factor: 2.459

7.  Simulations of oligomeric intermediates in prion diseases.

Authors:  David L Mobley; Daniel L Cox; Rajiv R P Singh; Rahul V Kulkarni; Alexander Slepoy
Journal:  Biophys J       Date:  2003-10       Impact factor: 4.033

Review 8.  Insights into Mechanisms of Transmission and Pathogenesis from Transgenic Mouse Models of Prion Diseases.

Authors:  Julie A Moreno; Glenn C Telling
Journal:  Methods Mol Biol       Date:  2017

9.  Testing the possibility to protect bovine PrPC transgenic Swiss mice against bovine PrPSc infection by DNA vaccination using recombinant plasmid vectors harboring and expressing the complete or partial cDNA sequences of bovine PrPC.

Authors:  Sandra Müller; Roland Kehm; Michaela Handermann; Nurith J Jakob; Udo Bahr; Björn Schröder; Gholamreza Darai
Journal:  Virus Genes       Date:  2005-03       Impact factor: 2.332

10.  Sheep-passaged bovine spongiform encephalopathy agent exhibits altered pathobiological properties in bovine-PrP transgenic mice.

Authors:  Juan Carlos Espinosa; Olivier Andréoletti; Joaquín Castilla; María Eugenia Herva; Mónica Morales; Elia Alamillo; Fayna Díaz San-Segundo; Caroline Lacroux; Séverine Lugan; Francisco Javier Salguero; Jan Langeveld; Juan María Torres
Journal:  J Virol       Date:  2006-11-01       Impact factor: 5.103
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