Daily Planet's Ingram discusses prion disease 0
Discovery Channel's Daily Planet co-host Jay Ingram visits Grande Prairie today to offer behind-the-scenes details of a mysterious and contagious series of diseases.
The lecture takes place at the Grande Prairie Regional College at 7 p.m., where Ingram discusses fatal prion diseases.
"Here at this very microscopic level, strange things are happening and just now we are beginning to figure out what they are," he said.
The most well-known form of a prion disease is bovine spongiform encephalopathy (BSE), widely known as the mad cow disease.
Prion diseases spread when malformed proteins attach themselves to healthy tissue. Unlike other infectious ailments, they are incurable.
"It is a protein that has gone wrong," said Stefanie Czub, a scientist with the University of Calgary and the Canadian Food Inspection Agency who will be present at the lecture.
"For other infectious diseases we have a cure or the body heals itself quite efficiently.prion diseases, once infected, they are invariably fatal."
A prion disease of current concern to Western Canadians is chronic wasting disease (CWD), affecting deer and elk in southern Saskatchewan and Alberta. Unlike mad cow disease, which infects the animal's brain, spinal cord and central nervous system, chronic wasting disease spreads to several parts of the animal, and is even present in urine and saliva.
Animals infected become very thin, and can carry the disease for two years before these signs become evident.
Prion diseases can only be formally diagnosed by sampling infected tissue.
"With all these diseases, it takes quite a long time for the symptoms to show," Ingram said. "You could hunt and kill a deer and eat it, and it might have the chronic wasting disease prions in it."
"The ultimate diagnosis can only be done on a piece of tissue, not in blood," Czub said.
Twenty deer have been identified in Alberta with CWD since monitoring of the disease began in 2005. While the cause of mad cow disease is generally believed to be the use of recycled beef and bone meal material in livestock feed, which became a common practice in the 1980s, CWD's cause remains unknown.
"Nobody really knows," Ingram said. "It could be that an infected deer goes to a salt lick, licks it, and the prions are in the saliva."
Ingram said that the disease is currently not an issue for the Peace Country, but infected deer and elk are bringing it into southern Alberta as they travel along the valleys of the South Saskatchewan and Red Deer Rivers.
"If chronic wasting disease spreads far enough north, especially in Saskatchewan, that it intersects with the caribou migration routes, and if caribou are susceptible, then you've got a huge problem on your hands," Ingram said.
The medical community is taking a close look at CWD due to the similarities prion diseases have with the degenerative Alzheimer's, Parkinson's and Lou Gehrig's diseases.
"The way that they spread is somewhat similar," Ingram said. "In Alzheimer's and Parkinson's and Lou Gehrig's disease, you get an accumulation in the brain of junk basically; they're called plaques, these sort of dark deposits if you look at brain tissue after autopsy."
"They are all part of the so-called protein-misfolding diseases," Czub said. "One might be a very good model for the other, so we need to keep that in mind. Especially with this enormous increase in Alzheimer's in the future to be expected. One in three over the age of 65 is going to develop Alzheimer's disease in the next 10 years."
Hosted by the Alberta Prion Research Institute, Ingram's lecture is open to the public free of charge in the Collins Recital Hall, room L106 today at 7 p.m.