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Discovery Channel host dissects BSE-causing prions

Unlocking the mystery behind BSE and chronic wasting disease could one day lead to a cure for Alzheimer’s, Parkinson’s and ALS an audience at Red Deer College heard Friday.
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Daily Planet host Jay Ingram was at Red Ceer College Friday night presenting the history of infectious proteins called prions

Unlocking the mystery behind BSE and chronic wasting disease could one day lead to a cure for Alzheimer’s, Parkinson’s and ALS an audience at Red Deer College heard Friday.

“There are certain features of these diseases that bear an uncanny resemblance to [BSE],” said Jay Ingram, host of Discovery Channel’s Daily Planet.

Ingram was the main speaker at The Prion Diaries at the Margaret Parsons Theatre. Ingram talked about the history and dangers BSE and chronic wasting disease still pose.

“These are not your average run-of-the-mill viruses,” Ingram said.

Misfolded proteins, called prions, cause BSE and chronic wasting disease in elk and deer. Unlike bacteria infections or diseases caused by viruses, there is currently no cure for treating animals or people infected with prions.

“With prions you don’t make any assumptions. We don’t know where it’s headed,” he said.

While BSE has been contained in Alberta since the initial discovery of a BSE-infected cow in 2003, Ingram warned that chronic wasting disease, found in wild deer and elk, poses a threat.

While BSE was transmitted by the ingestion of protein and bone meal of infected animals, chronic wasting disease can be transmitted through the saliva, urine, and feces of deer and elk.

“Prions can last ten years in the soil,” Ingram said.

“If you’re a deer hunter is it safe to eat venison? We don’t know,” he added.

Game hunters and people who eat venison can take solace in the fact that chronic wasting disease doesn’t pose nearly the same health threat to humans.

“It’s quite difficult for chronic wasting disease prions to kill humans,” he said.

In the aftermath of the discovering of BSE in Alberta in 2003, the federal and provincial governments jointly funded the establishment of Prionet Canada, a government research institute dedicated to looking at how to deal with prions.

For proteins to work, they must be folded in a specific three-dimensional shape. It is when normally inert prions undergo a misfolding that they pose a threat.

“These misfolded proteins can migrate and spread throughout the brain,” Ingram said.

For more information on prion diseases visit PrioNet’s website at www.prionetcanada.com.