Chronic wasting disease prions can convert human prions into disease-associated form

Jul 2018: A team of CCBS researchers led by Marcelo Barria have published findings that prions that cause chronic wasting disease in deer and elk can convert human prions in a test tube to the disease-associated form.

Chronic wasting disease (CWD) is a contagious and fatal neurodegenerative disease and a serious animal health issue for deer and elk in North America. It might also be a public health issue. The recent discovery of CWD among free-ranging reindeer and moose in Europe prompted a group of CCBS researchers at the National CJD Research and Surveillance Unit to revisit the unresolved issue of whether CWD can spread from animals to humans, as can bovine spongiform encephalopathy (so-called mad cow disease).

Both diseases are caused by prions, misfolded protein particles that are detected in the neural tissue of infected animals. The researchers, funded by the Department of Health and Social Care and the Scottish Government, found that CWD prions from cervids (e.g., elk, white-tailed deer, and reindeer) in North America can convert the human brain prion protein in a test tube into the disease-associated form but that the efficiency of conversion is affected by variation in the cervid and human prion protein genes.

Given the similarity of North American cervids to those in Europe, the researchers underscore the need for a more comprehensive and thorough assessment of whether CWD can cross the species barrier and infect humans.

The findings have been published today in the journal Emerging Infectious Diseases. "Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions" by Marcelo A. Barria, Adriana Libori, Gordon Mitchell & Mark W. Head.

Chronic wasting disease prions can convert human prions into disease-associated form

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