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Prions have traditionally been linked with the development of Creutzfeld-Jakob Disease (CJD), the brain-wasting equivalent of mad cow disease (also known as bovine spongiform encephalopathy).

Prions — infectious agents that cause diseases like the human variant of mad cow disease — also have protective properties, new research suggests.

When functioning normally, prion proteins protect neurons in the brain from becoming overstimulated and dying, indicates the study, published in the May 5 issue of the Journal of Cell Biology.

Prions have traditionally been linked with the development of neurodegenerative diseases like Creutzfeld-Jakob Disease (CJD), the brain-wasting equivalent of mad cow disease (also known as bovine spongiform encephalopathy, or BSE).

In this role, abnormal prions cause plaques to form on the neurons preventing them from functioning properly.

Researchers at Rockefeller University discovered that when they removed prion proteins from the brain cells of mice, their neurons overreacted to electrical and drug-induced stimulation, eventually dying.

The authors believe that prion proteins only turn deadly when they are physically altered, as they can no longer regulate the behaviour of the neurons and offer a neuroprotective effect. Researchers aren't sure how this transformation occurs.

In other news, PrioNet Canada, a network of research groups that aims to eradicate prion diseases, announced Wednesday it has awarded $8 million to Canadian researchers to help prevent prion-related diseases.

The funding will support 19 projects across the country and involve 60 Canadian scientists dedicated to researching BSE, CJD, a variant human form of CJD acquired from eating BSE-contaminated cattle products and chronic wasting disease (CWD) in deer and elk.

"Ultimately, these projects will translate to safer food, health, and environmental systems for Canadians," said Dr. Neil Cashman, scientific director of PrioNet, in a release.

The projects announced Wednesday include:

  • The development of a BSE vaccine for cattle to ensure that Canadian herds are protected against BSE.
  • A strategy to minimize the spread of CWD among deer and elk.
  • The development of new food testing standards for BSE.