The prion protein to blame for BSE has a shadow, or Shadoo, as scientists are calling their new discovery.
Until recently the PrP protein that potentially folds over and accumulates in the brains of animals, causing transmissible spongiform encephalopathy diseases, was thought to be unique.
It turns out that it has a mysterious cousin, the Shadoo.
Shadoos can be found in the brain until such time as PrPs begin to fold over and clump together, leading to diseases such as BSE that cause brain cells to die.
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“Then it disappears,” said University of Alberta researcher David Westaway.
Westaway’s team at the U of A, along with researchers in Montana, Ontario and Ohio, initially discovered the chemical effects of a prion rather than the Shadoo itself.
“We could tell there was something there. We have thought there was something else present since 1996. We just had to find it,” he said.
Joel Watts of the University of Toronto is the lead author of the paper that announced the discovery.
He said the Shadoo prion, identified in a mouse model, shares similar structure to healthy PrP.
Westaway said the research hasn’t proven that the new prion is related to disease-causing TSEs, but it is likely.
The discovery of the tiny, elusive protein is monumental in prion research, said scientists working on diseases such as BSE in cattle, scrapie in sheep, chronic wasting disease in deer and elk and Creutzfeldt-Jakob disease in humans.
“We believe normally the Shadoo has a protective role for neurons in the brain,” said Westaway.
Watts said the Shadoo was present mainly in areas of the brain where the healthy PrP was absent. This suggests the Shadoo might offer protection to neurons in the absence of healthy PrPs.
But the Shadoo may have a dark side. Westaway said the new protein might also be changing shape, like the PrP, and disappearing as it begins to take part in causing disease.
Watts, Westaway and their colleagues are now working to make the Shadoo prions fold and become misshapen in hopes this might give them a lead in finding a target for drugs that would combat the disease.
Westaway said the discovery is akin to finding a continent if considered in geographic terms.
“This isn’t the discovery that says we have a cure or even the one where we announce we have identified (the cause of protein folding). But it is a critical piece of the puzzle.
“This should give farmers hope that research is closing in on some answers about these diseases.”
