Strain features and seeding properties by Protein Misfolding Cyclic Amplification of LBSE and sporadic CJD (type 2) prion strains following disease transmission in an ovine transgenic mouse model with co-expression of a resistance allele (R171)
2014 : 2 years / Subvention : 30 000 euros
Project: The objective of this project wasto study and characterize the transmission of the atypical L-BSE strain and sporadic CJD (type 2) previously adapted to Q171 ovine transgenic mice (TgOvPrP4) in ovine transgenic mice heterozygousfor a resistance prion gene allele (RQ171), after breeding with a Q171 ovine transgenic mouse line (P10.36). Classical parameters analysis (incubation time, lesion profiles, molecular profiles) and PMCA assays were proposed to study these transmissions.
Main results and related published data: A panel of strains is being investigated by bioassays that are still going on, including sporadic CJD types 2 and 1, L-BSE but also scrapie sources CH1641 and 87V, in Q171 (TgOvPrP4), RQ171 (TgOvPrP4 x P10.36) and R171 (P10.36) mouse lines. QR171 ovine transgenic mice are, as expected, resistant to most of these prion strains, but our results alreadyshow thatsome strainscan unexpectedly be adapted and transmit even in R171 mice. PMCA results are overall consistent with current results of bioassays.