Prion diseases animal models

Human prion disease models have also been developed in mice [154,155]. Crossing the species barrier into an experimentally accessible animal system, the prions responsible for Creutzfeldt Jakob disease, new variant CJD, Gerstmann-Straussler-Scheinker disease, and fatal familial insomnia produce a reproducible time-dependent neuronal degeneration leading to death. 

Abstract Here we review the known strain profiles of various prion diseases of animals and humans, and how transgenic mouse models are being used to elucidate basic molecular mechanisms of prion propagation and strain variation and for... 

Although PrP-res is associated with TSE infectivity and has, at the biochemical level at least, some limited self-propagating activity, important questions remain about the PrP-only prion model for the TSE agent. For instance, why has no one been able to demonstrate the model s most basic prediction, that is, that PrP-sen alone can be induced to convert to an infectious agent that causes TSE disease when inoculated into animals Inoculation of a synthetic PrP peptide has induced a TSE-like disease in transgenic mice expressing a familial TSE associated mutant PrP, but the serial transmissibility of this disease remains to be determined (Kaneko et al, 2000). Why are 100,000 PrP-res molecules present per infectious unit Why is PrP-res not always found in infectious tissues (Lasmezas et al, 1997) Why are nucleic acids (Akowitz et al,... 

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