Scientists Show In Vitro Spontaneously Misfolded Proteins Can Cause Spongiform Encephalopathy in Mice

Jan. 25, 2017

Washington, DC – January 25, 2017 – Chronic Wasting Disease (CWD) is the only known transmissible spongiform encephalopathy (TSE) that affects free-ranging wildlife, specifically cervids such as elk, deer, moose, caribou and reindeer. CWD has become endemic in both free-ranging and captive herds in the North America, South Korea and, most recently, northern Europe. 

The prion research community continues to debate the origins of CWD. Original foci of CWD emergence in Colorado and Wyoming coincident with the sheep TSE scrapie, suggest that scrapie prions may have adapted to cervids to cause CWD. However, emerging evidence supports that cervid prion protein may be more prone to misfolding to the pathologic isoform. The researchers tested the hypothesis that cervid prion protein can spontaneously misfold to create de novo prions. Whether CWD can arise spontaneously as a sporadic TSE or represents a new TSE caused by cervid-adapted scrapie prions profoundly impacts surveillance and mitigation strategies.

The full study is published today in mSphere, an open-access journal of the American Society for Microbiology.