Project 3B: Pathogenesis Transmission and Detection of Zoonotic Prion Diseases

Project Summary:
Chronic wasting disease (CWD) is an emergent, highly transmissible, geographically expanding, prion
disease of both wild and captive cervids. CWD is unique among prion diseases in its facile contagion and
environmental persistence. Its expanding geographical range, combined with the increasing transport of
animals and animal products, portend its continued expansion and diversification. The zoonotic potential of
CWD remains poorly understood. CWD endemic areas interface cervids with livestock species and humans,
posing obvious zoonotic risks that over time will increase. While it is known that strains of CWD exist, nothing
is known about the zoonotic potential of these strains. Work from our applicant group has shown that CWD-
infected cervids continually shed prions into the environment and that previously unrecognized environmental
factors can influence the emergence of a dominant strain from a mixture. The ability to recognize the zoonotic
potential of CWD strains is central to mitigating CWD transmission risk. The central hypothesis for work
described here is that CWD strains evolve continuously due to a combination of both host and environmental
factors. We will test this hypothesis by: i) determining the evolution and zoonotic impact of CWD strains in the
native cervid species; ii) leveraging our unique animal resources, expertise, and in vivo & in vitro
methodologies to assess environmental factors that alter CWD strain selection and evolution and iii) evaluate
zoonotic potential of CWD strains by a complementary combination of in vitro amplification assays and
animal transmission studies. The results will provide new information about this emergent transmissible prion
disease and the risk it poses to humans and other species.

Grant Number: 5P01AI077774-14
NIH Institute/Center: NIH
Principal Investigator: Jason Bartz