Sc237 hamster PrPSc and Sc237-derived mouse PrPSc generated by interspecies in vitro amplification exhibit distinct pathological and biochemical properties in tga20 transgenic mice

作者: Miyako Yoshioka , Morikazu Imamura , Hiroyuki Okada , Noriko Shimozaki , Yuichi Murayama

DOI: 10.1111/J.1348-0421.2011.00328.X

关键词:

摘要: Prions are the infectious agents responsible for transmissible spongiform encephalopathy, and primarily composed of pathogenic form (PrP(Sc)) host-encoded prion protein (PrP(C)). Recent studies have revealed that misfolding cyclic amplification (PMCA), a highly sensitive method PrP(Sc) detection, can overcome species barrier in several xenogeneic combinations seed PrP(C) substrate. Although these findings provide valuable insight into origin diversity prions, differences between generated by interspecies PMCA vivo cross-species transmission not been described. This study investigated histopathological biochemical properties brains tga20 transgenic mice inoculated with Sc237 hamster scrapie from Sc237-derived mouse PrP(Sc), which had using as normal brain homogenate Tga20 overexpressing were susceptible to after primary transmission. differed their lesion profiles accumulation patterns, Western blot profiles, denaturant resistance. In addition, exhibited distinctive virulence upon secondary passage. These results suggest different vitro environments result propagation biological properties.

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