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Prion Find Brings Insights Into Brain-Wasting

17 August 2007

EDMONTON - Researchers have discovered a new prion protein that could bring fresh insights into how brain-wasting disease develops in animals and humans.

A BSE infected cow digging to nowhere.

This is the first discovery of a new brain prion protein since 1985. Called Shadoo - a shadow of the well-known prion protein called PrP - the new protein may protect brain cells from disease in some circumstances, early experiments suggest.

But David Westaway, a professor at the University of Alberta who worked with researchers from Toronto, Montana and Ohio to make the discovery, said a fuller study needs to be done to see if the new protein also has a bad side.

"This is not a miracle cure type of discovery," said Westaway, director of the Centre for Prions and Protein Folding Diseases at the U of A. "This is a pure science discovery. In the world of prion biology, it's discovering a new continent. We didn't even know this metaphorical continent was there. It means we were missing a piece of the puzzle. It means, in the longer term, that we may understand better why we have normal prion proteins in our body and it may help us understand when prion infections affect animals, why the brain cells get killed."

Until now, scientists focused their research on what they thought was the unique prion protein called PrP that at first protects healthy brain cells, but then improperly folds into itself. That folding or deformation causes mad cow or bovine spongiform encephalopathy in cattle, scrapie in sheep, chronic wasting disease in mule deer and elk, and Creutzfeldt-Jakob disease in humans.

Westaway said more research is needed to determine if Shadoo also changes and folds in on itself, essentially breeding infection where it once protected cells from that infection.

"It has a protective function for brain cells, but one implication of our finding is we have to look into whether it has a bad side, whether this (Shadoo) can change shape and do bad things as well," said Westaway, whose research is published in the European Molecular Biology Organization Journal. Westaway said experiments show the new protein disappears when disease takes over.

"In the big scheme of things, it's a piece of the puzzle we've been missing," Westaway said.

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Source: EdmontonJournal


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