More Prion Study; They Dont Cook Out

Couple tackles cause of Creutzfeldt-Jakob

Mon, December 29, 2008

Marco and Vania Prado are researching a class of always fatal brain diseases in hopes of coming up with treatments and cures

By JOHN MINER / London Free Press

Molecular neuroscientists Marco and Vania Prado hope by studying the good they can understand the bad.

Recruited this fall to Robarts Research Institute from a Brazilian university along with their seven-member research team, the couple is tackling an untreatable and always fatal class of brain diseases -- prion disease.

"What we know is there is a protein that every one of us has called the prion protein, but we don't really know what the function is. It changes, it goes bad," said Marco, who has received nearly $450,000 over two years from PrioNet Canada, a network of researchers, for his research in London.

Vania's expertise is in developing lines of mice for research that display degenerative diseases.

When the prion protein goes bad in humans, it causes the degenerative Creutzfeldt-Jakob disease.

It is believed prion disease has been present in sheep as scrapie for hundreds of years, but didn't become a major health threat until it somehow made the leap to cattle and sparked the mad cow epidemic in Britain.

The discovery of an infected cow in Alberta in May 2003 shut down Canada's livestock export industry, costing farmers billions of dollars and crippling the beef industry.

Not a bacteria or virus, prions have turned out to be a particularly difficult infectious agent to kill, defying normal sterilization procedures for surgical instruments. Cooking won't kill prions in food.

When a surgery patient in London initially tested positive for Creutzfeldt-Jakob disease in 2006, London Health Sciences Centre was forced to shut down all its operating rooms for several days in order to prevent the disease from being spread.

"It is one of the worst things to kill. It can survive bound to metals in the environment for many, many years," Marco said. "The prion protein has a form, a conformation. When that conformation changes, it causes disease.

"It may take a long time. It may take 20 to 40 years for the disease to show up, but you get the disease if that conformation is changed."

With only about one in one million people developing the disease, it was once considered a pretty obscure area for research, he said.

Fortunately, some scientists were intrigued enough to study prions so there was enough understanding for health authorities to halt the outbreak in Britain, Marco said.

The London research team plans to study "good prions" to gain an understanding of what prions actually do in the brain.

"First you have to understand how it works well to understand what goes bad in the disease," Marco said.

London Free Press;
http://lfpress.ca/newsstand/News/Local/2008/12/29/7870731-sun.html