Detection of the disease-associated form of the prion protein in biological samples

Transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases that occur in a variety of mammals. In TSEs, a chromosomally encoded protein (PrPC) undergoes a conformational change to the disease-associated form (PrPd). PrPd is capable of inducing a change in additional molecules of PrPC to the PrPd conformation. TSEs are inevitably fatal and cross-species transmission is known to occur, and there is potential for transmission via blood transfusion and organ transplantation in humans. Thus, there is interest in high-quality diagnostics for both humans and animals. This review summarizes methods of TSE detection currently in use in diagnostic settings and discusses recent advances in PrPd detection that afford substantial enhancements in sensitivity over currently approved methods for use in clinical settings.