Fig. 1. Generation of misfolded prions in TSEs.

PrP^Sc, the disease-associated form of the prion protein, is able to impose its structure to a normally produced protein, termed PrP^C. As result, an exponential generation of misfolded prions is obtained. PrP^Sc has been associated with several cytotoxic events in vitro and in vivo. The accumulation of the abnormal protein in the brain leads to clinical decline, which invariably ends in the death of the affected individuals.