Chronic wasting disease (CWD) is a cervid (deer, elk, moose, etc.) prion disease that is readily transmitted among wild and farmed cervids and acquired from CWD-contaminated environments. CWD-infected cervids have been found in 26 states, 3 Canadian provinces (Alberta, Quebec, and Saskatchewan) and CWD was inadvertently exported to South Korea. An independent outbreak of CWD was observed in Scandinavian (Norway, Finland, and Sweden) moose, reindeer, and red deer. The CWD pathology propagates by inducing a natively expressed prion protein (PrPC) to adopt the CWD prion conformation (PrPSc). PrPC and PrPSc differ solely in their respective conformations. Like other prion diseases, CWD is both sporadic and transmissible. The CWD conformation can shift in response to natural selection pressures resulting in new CWD strains with differing phenotypes. CWD alters cervid population genetics, which, in the future, may result in the emergence of novel CWD strains. Millions (11.5) of Americans hunt and annually harvest nearly 6 million deer, indicating that CWD represents an underappreciated potential threat to a significant segment of the American food supply. Should a zoonotic CWD strain emerge, it could negatively impact American hunters and consumers of game meats.

Challenges of detecting an evolving prion disease, chronic wasting disease (CWD)

ACS Spring 2022

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