Submitted to: Book Chapter
Publication Type: Book / Chapter
Publication Acceptance Date: October 29, 2013
Publication Date: N/A
Technical Abstract: Prion diseases or transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases that affect several species of animals and include bovine spongiform encephalopathy (BSE), scrapie in sheep and goats, chronic wasting disease (CWD) in cervids, and transmissible mink encephalopathy. These chronic diseases are associated with the accumulation of a protease-resistant, disease-associated isoform of the prion protein, called PrP**Sc, in the central nervous system and other tissues, depending on the host species. In most instances, the TSEs of animals are acquired through exposure to infectious material, but inherited and spontaneous TSEs also occur. These diseases share pathologic features and infectious mechanism, but have distinct differences in transmission and epidemiology due to host factors and strain differences encoded within the structure of the misfolded protein. The unique mechanism of prions and the possibility that BSE can be transmitted to humans in the form of variant Creutzfeldt-Jakob disease has brought attention to this family of diseases.