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Collinge, J. & Clarke, A. R. A general model of prion strains and their pathogenicity.
Using the ME7 model of prion disease, Cunningham and colleagues demonstrated that the primed brain showed exaggerated response to several challenges, including LPS, poly I C, IL-1β, and TNF-α [89 91].
However, a model of prion replication must explain both how prions propagate, and why their spontaneous appearance is so rare.
Tissues from the RML murine model of prion pathogenesis were collected at various stages throughout pathogenesis.
C57Bl/6 mice inoculated with the Me7 prion strain are an effective model of prion diseases that have been well described for their incubation time, vacuolization profiles, and immunohistology [34], [35].
Observed prevalence was relatively high compared to that previously reported in mule deer populations elsewhere [6], [17], but still within the range predicted by a simple model of prion epidemic dynamics [8].
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The amino acid frequency propensities obtained from the known PrD training dataset described above were used to build an independent log-likelihood model of prion-forming domains.
Modeling of prion diseases has contributed and will continue to contribute to the understanding of the pathogenesis as well as of the therapeutics of these diseases, but crucial experiments remain elusive.
The peripheral distribution of PrPSc has been well characterized in rodent models of prion diseases [32], [33].
This fact is also known from rodent models of prion infection [31], but had never been demonstrated in primates before.
To ascertain whether LSPC knockdown of PrPC expression sufficed to cure prion infection, we used two cell culture models of prion infection.
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