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Finding a way forward in the fight against prion disease


Valerie Sim, an assistant professor in the University of Alberta's Faculty of Medicine & Dentistry, and Leonardo Cortez, a research associate in her lab, examining the use of two bile acids as possible therapeutic treatments for prion disease. The research found that ursodeoxycholic acid (UDCA) and tauroursodeoxycholic acid (TUDCA) helped slow the progression of the disease if given early in the disease process. The bile acids appear to bind the proteins that cause disease and prevent them from spreading.

"These compounds are normally used to help digest lipids and fats, but interestingly they've been used in naturopathic and Chinese remedies for hundreds of years," says Sim. "Right now we have nothing to offer patients with prion disease. This could be a way forward."

While Sim's research marks important new progress, she cautions it needs further study in humans. She also notes that the use of UDCA and TUDCA would not be effective in most cases of prion disease as patients only come to medical attention after the disease is already too far progressed. She does believe the findings could have application for the 10 per cent of Creutzfeldt-Jacob patients who have a genetic form of the disease and who could seek early long-term treatment.

"Some of those people know they are carriers of the disease and currently we have nothing we can give them that works," says Sim. "This is not a cure, but may have some benefit if given early. And for these patients, any benefit is better than nothing."

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