Prion diseases or transmissible spongiform encephalopathies (TSEs) are fatal neu-rodegenerative disorders (198). The crucial event in the development of these diseases is the conformational change of a membrane bound protein, the cellular PrPC in Figure 3.1, into a disease associated, bril-forming isoform (199). Despite their rare incidence, TSEs have captured very large attention from the scienti c community due to the unorthodox mechanism by which prion diseases are transmitted...
