Interspecies transmission to bovinized transgenic mice uncovers new features of a CH1641-like scrapie isolate 07 Agricultural and Veterinary Sciences 0707 Veterinary Sciences

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Abstract

In animal prion diseases, including bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease in cervids, and scrapie in sheep and goats, a disease-associated isoform of prion protein (PrPd) accumulates in the brains of affected animals. Although the CH1641 scrapie isolate was experimentally established in the UK, a few natural CH1641-like scrapie cases have been reported in France and the UK. The molecular mass of the unglycosylated protease-resistant core of PrPd (PrPres) is known to be similar between CH1641-like scrapie and experimental BSE in sheep. We previously established an experimental CH1641-like scrapie isolate (Sh294) from a natural classical scrapie case. Here, we demonstrated that the Sh294 isolate was independent of both classical and atypical BSEs by cross-species transmission to bovine PrP overexpressing (TgBoPrP) mice and wild-type mice. Interestingly, we found that the Sh294 isolate altered its host range by the transmission to TgBoPrP mice, and we succeeded in the first stable reproduction of CH1641-like scrapie specific PrPres banding patterns with the ∼12-kDa small C-terminal fragment in wild-type mice. This study provides new insight into the relationship between CH1641-like scrapie isolates and BSEs. In addition, interspecies transmission models such as we have demonstrated here could be a great help to investigate the origin and host range of animal prions.

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Miyazawa, K., Masujin, K., Matsuura, Y., Iwamaru, Y., Yokoyama, T., & Okada, H. (2018). Interspecies transmission to bovinized transgenic mice uncovers new features of a CH1641-like scrapie isolate 07 Agricultural and Veterinary Sciences 0707 Veterinary Sciences. Veterinary Research, 49(1). https://doi.org/10.1186/s13567-018-0611-1

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