Prion diseases like BSE have a telltale sign

The discovery suggests the disease may not be 
as unstoppable as previously thought

Medical researchers at the University of Alberta have found fatal prion diseases, like BSE, have a hidden signature.

Findings published this month in the peer-reviewed journal, Public Library of Science (PLoS) Pathogens, show that up to seven months before an animal shows physical signs of infection, a particular prion protein in the brain, known as shadoo protein, was being eradicated.

“What we discovered is that as the early prion disease process unfolds in an infected brain, that the shadoo protein is simultaneously disappearing,” lead author David Westaway, a researcher in the faculty of medicine and dentistry at the University of Alberta, said.

“This is telling us there is a process we were previously unaware of, a process that is happening before the infected animals are getting sick.”

Westaway says this likely means the brain cells are trying to defend themselves against the disease, and could mean it’s not unstoppable.

“There could be a cellular process trying to destroy the infectious prions as they appear. And if we could help that process a little bit more, that might be an avenue to attenuate the disease,” Westaway said.

Westaway collaborated with a team of researchers from Ontario, the University of California, the Institute for Systems Biology in Washington, the McLaughlin Research Institute in Montana and a researcher in Germany, on this discovery.

The next step for Westaway’s research team is to determine why this shadoo protein is disappearing.

“We need to better understand this. We want to solve this mystery,” he said.

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