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Volume 19, Number 12—December 2013


Spontaneous Generation of Infectious Prion Disease in Transgenic Mice

Juan-María TorresComments to Author , Joaquín Castilla, Belén Pintado, Alfonso Gutiérrez-Adan, Olivier Andréoletti, Patricia Aguilar-Calvo, Ana-Isabel Arroba, Beatriz Parra-Arrondo, Isidro Ferrer, Jorge Manzanares, and Juan-Carlos Espinosa
Author affiliations: Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria, Madrid, Spain (J.-M. Torres, J. Castilla, B. Pintado, A. Gutiérrez-Adán P. Aguilar-Calvo, A.-I. Arroba, B. Parra-Arrondo, J.-C. Espinosa); Basque Foundation for Science, Bilbao, Spain (J. Castilla); Ecole Nationale Vétérinaire de Toulouse, Toulouse, France (O. Andréoletti); Hospitalet de Llobregat, Barcelona, Spain (I. Ferrer); Universidad Miguel Hernandez, Sant Joan d´Alacant, Spain (J. Manzanares)

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Table 1

Onset of clinical signs and survival times for transgenic mice expressing different levels of mutant 113LBoPrP or wild-type BoPrP*

Transgenic mouse line Transgene expression level† Onset of clinical signs, days ± SEM (no. diseased/no. tested) Death, days ± SEM
113LBoPrP-Tg009+/–    0.5×     >550 (0/9)   >550
113LBoPrP-Tg009+/+    1×     >550 (0/10)   >550
113LBoPrP-Tg037+/–    3×     272 ± 33 (10/10)   345 ± 49
113LBoPrP-Tg037+/+    6×     187 ± 18 (6/6)   223 ± 47
BoPrP-Tg110+/–    4×     >550 (0/6)   >550
BoPrP-Tg110+/+    8×     >550 (0/10)   >550
Non-Tg (Prnp–/–)    0×     >550 (0/9)   >550

*BoPrP, bovine prion protein; 113L, leucine substitution at codon 113; +/–, hemizygous for bovine prion protein (Prnp) gene; +/+, homozygous for bovine Prnp gene. All transgenic animals were murine Prnp−/−.
†Relative to cattle PrP expression.
‡This BoPrP transgenic mouse line has been described (20,22).

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