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Volume 19, Number 12—December 2013
Research

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)

Main Article

Table 2

Infectivity of 113LBoPrP-Tg037 mice brain homogenates in BoPrP-Tg110*

Inoculum Mean ± SEM survival time, days (no. diseased/no. tested)†
First passage Second passage Third passage
113LBoPrP-Tg037 (179 d old)‡ 330 (1/5) 272 ± 38 (6/6) 252 ± 23 (6/6)
113LBoPrP-Tg037-pool (150–250 d old)§ 322, 406 (2/6) 291 ± 23 (6/6) 255 ± 14 (7/7)
wtBoPrP-Tg110-pool (500–600 d old) >650 (0/12) >650 (0/12) NA
Normal mouse brain (500–600 d old) >650 (0/12) >650 (0/12) NA
Classical BSE-C (VLAPG817/00) ¶ 295 ± 12 (6/6) 265 ± 35 (6/6) NA
Atypical BSE-H (CASE 03–2095)# 292 ± 5 (6/6) 296 ± 7 (6/6) NA
Atypical BSE-L (02.2528)** 207 ± 7 (6/6) 199 ± 1 (6/6) NA

*BoPrP, bovine prion protein; 113L, leucine substitution at codon 113; wtBoPrP, wild-type bovine prion protein; NA, not available; BSE, bovine spongiform encephalopathy.
†Intracerebral inoculation with 2 mg brain tissue equivalent in BoPrP-Tg110+/+ (8× expression level).
‡Brain homogenate from a unique terminally sick animal.
§Brain homogenate from a pool of 5 terminally sick mice that died at 150–250 d of age.
¶Brainstem homogenate of 1 cow naturally infected with classical BSE (RQ 225:PG817/00) supplied by the Veterinary Laboratories Agency (New Haw,
#Data from Torres et al. (31).
**Brainstem samples from naturally affected cows given a diagnosis of atypical BSE-L by the Agence Française de Sécurité Sanitaire des Aliments (Lyon, France).

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