Assessment and comparison of the barriers to interspecies transmission of bovine spongiform encephalopathy and chronic wasting disease prions
DOI:
https://doi.org/10.21423/aabppro20153580Keywords:
prion disease, epidemiology, species barriersAbstract
Prion diseases are transmissible, neurodegenerative diseases caused by templated conversion of the normal cellular prion protein to a misfolded, neurotoxic form. After conversion, the misfolded protein aggregates into characteristic protease-resistant amyloid plaques, which are hallmarks of prion disease. Species barriers among prion diseases have been observed epidemiologically and experimentally, but the mechanism(s) mediating this phenomenon remains unknown, making it impossible to predict the risk of transmission to new species, including humans, and to formulate therapies and prophylactics... Functional demonstration of the mechanism(s) resulting in the species barriers is the principal question addressed in this research.