Bile Acids Could Be Used to Treat CWD

Bile acids could help slow the progression of a rare and fatal neurodegenerative disease, research from the University of Alberta has found.

Prion disease occurs when a protein changes into an odd shape and causes normal proteins in its path to do the same. For reasons scientists don’t quite understand, brain cells then start dying.

The disease affects one or two people out of every million each year and is known as mad cow disease in cattle and chronic wasting disease in deer and elk.

Valerie Sim, a clinician-scientist at the University of the Alberta, said she was “somewhat skeptical” when a research associate in her lab suggested they test bile acids as a potential cure. But that hunch eventually led to a paper published in August in the Journal of Virology. Leonardo Cortez, her research associate, is listed as first author.

Ursodeoxycholic acid (UDCA) and tauroursodeoxycholic acid (TUDCA) are natural compounds made by the liver and used by the body to help break down fats. But they have also been known to protect against cell death for several other neurodegenerative diseases. TUDCA, for example, has been shown to slow the progression of ALS.

To test the acids’ effectiveness, researchers placed slices of mice brain on a dish, infected them with prions and then treated them with the acids.

Surprisingly, they observed that the acids seemed to bind to the “promiscuous proteins” and partially stop them from spreading. The acids also helped reduce brain cell death.

This potential cure could mean the most for the 10 per cent of prion disease patients who inherit it genetically, Sim said. The treatment appears to be most effective when administered early, long before symptoms even appear.

Their research also contributes to our understanding of other diseases (such as Alzheimer’s, Parkinson’s and Huntington’s) caused by problem-proteins.

Sim and her colleagues are now focused on testing combinations of compounds that might be even more effective at blocking the protein conversion and preventing cell death.

“I don’t think one compound will ever treat any of these diseases,” Sim said. “We hope we can find a combination.”