Efficient transmission of two different sheep scrapie isolates in transgenic mice expressing the ovine PrP gene

Journal of Virology
C CrozetT Baron

Abstract

We produced transgenic mice expressing the sheep prion protein to obtain a sensitive model for sheep spongiform encephalopathies (scrapie). The complete open reading frame, with alanine, arginine, and glutamine at susceptibility codons 136, 154, and 171, respectively, was inserted downstream from the neuron-specific enolase promoter. A mouse line, Tg(OvPrP4), devoid of the murine PrP gene, was obtained by crossing with PrP knockout mice. Tg(OvPrP4) mice were shown to selectively express sheep PrP in their brains, as demonstrated in mRNA and protein analysis. We showed that these mice were susceptible to infection by sheep scrapie following intracerebral inoculation with two natural sheep scrapie isolates, as demonstrated not only by the occurrence of neurological signs but also by the presence of the spongiform changes and abnormal prion protein accumulation in their brains. Mean times to death of 238 and 290 days were observed with these isolates, but the clinical course of the disease was strikingly different in the two cases. One isolate led to a very early onset of neurological signs which could last for prolonged periods before death. Independently of the incubation periods, some of the mice inoculated with this isolate sh...Continue Reading

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Citations

Oct 18, 2002·Trends in Molecular Medicine·Thierry Baron
Jun 18, 2002·APMIS : Acta Pathologica, Microbiologica, Et Immunologica Scandinavica·Michael A Tranulis
Apr 20, 2007·Journal of Virology·Thierry Baron, Anne-Gaëlle Biacabe
Nov 11, 2006·Annual Review of Genetics·Stanley B Prusiner, Maclyn McCarty
Feb 1, 2008·Annual Review of Pathology·Adriano AguzziMathias Heikenwaelder
Apr 13, 2006·PLoS Pathogens·Joel C WattsDavid Westaway
Nov 10, 2013·PLoS Pathogens·Eri SaijoGlenn C Telling
Apr 9, 2008·Emerging Infectious Diseases·Stéphanie SimonJacques Grassi
Jun 1, 2006·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·Stéphane LezmiThierry Baron
Feb 9, 2012·Experimental and Molecular Pathology·Alana M ThackrayRaymond Bujdoso
Nov 29, 2011·Experimental and Molecular Pathology·Alana M ThackrayRaymond Bujdoso
Nov 28, 2015·The Journal of General Virology·Lorenzo GonzálezMartin Jeffrey
Jul 21, 2005·The European Journal of Neuroscience·Michel DronFrançoise Dandoy-Dron
Feb 19, 2009·Journal of Neurochemistry·Fiona E ParkinsonMary Lynn Duckworth
Nov 15, 2008·The Journal of General Virology·Michele Angelo Di BariRomolo Nonno
Mar 23, 2012·The Biochemical Journal·Alana M ThackrayRaymond Bujdoso
Apr 8, 2003·The Journal of Veterinary Medical Science·Altangerel GombojavMasuhiro Takata
Feb 27, 2009·Prion·Samuel E SaundersJason C Bartz
Oct 16, 2010·Prion·Kevin C Gough, Ben C Maddison
Mar 16, 2007·Histochemistry and Cell Biology·Anna BencsikThierry Baron
Feb 21, 2008·Veterinary Research·Martin H Groschup, Anne Buschmann
Jun 4, 2008·Veterinary Research·Vincent BéringueHubert Laude
Oct 16, 2004·The Journal of General Virology·J-Y MadecT Baron
May 25, 2002·Journal of Neuropathology and Experimental Neurology·Thierry Baron
Nov 14, 2006·The Journal of General Virology·Giuseppe LaFauciRichard Rubenstein

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