Abstract

Chronic wasting disease (CWD) is a fatal, transmissible prion disease that affects captive and free-ranging deer, elk, and moose. Although the zoonotic potential of CWD is considered low, identification of multiple CWD strains and the potential for agent evolution upon serial passage hinders a definitive conclusion. Surveillance for CWD in free-ranging populations has documented a continual geographic spread of the disease throughout North America. CWD prions are shed from clinically and preclinically affected hosts, and CWD transmission is mediated at least in part by the environment, perhaps by soil. Much remains unknown, including the sites and mechanisms of prion uptake in the naive host. There are no therapeutics or effective eradication measures for CWD-endemic populations. Continued surveillance and research of CWD and its effects on cervid ecosystems is vital for controlling the long-term consequences of this emerging disease.

Highlights

  • Chronic wasting disease (CWD) is a fatal, transmissible prion disease that affects captive and free-ranging deer, elk, and moose

  • CWD was identified in the late 1960s and recognized as a spongiform encephalopathy by Williams in 1980 [1]

  • The infectious agent of CWD is the abnormally folded prion protein designated PrPSc, which is distinguished from the normal cellular prion protein (PrPc) by its resistance to proteolysis, propensity for aggregation, and insolubility in detergents [4]

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Summary

Introduction

Chronic wasting disease (CWD) is a fatal, transmissible prion disease that affects captive and free-ranging deer, elk, and moose. CWD continues to emerge and spread in free-ranging and captive cervids throughout the United States and Canada. In most locations reporting CWD cases in free-ranging animals, the disease continues to emerge in wider geographic areas, and prevalence appears to be increasing in many diseaseendemic areas.

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