Simple Chemical Stops Prion Disease

Mad cow, scrapie and Creutzfeld-Jakob disease are all diseases of the brain that debilitate before they kill, and have no cure, or even many good treatment options beyond supportive care. But now, researchers in Switzerland say it's possible to block the misfolded proteins called prions that cause these diseases, by using a compound that biologists have used to track other molecules.

Prions are a distinct type of protein — they can self-replicate, said Adriano Aguzzi, professor of neuropathology at the University of Zurich, who led the study. When a prion comes into contact with a normal protein, it makes the normal protein alter its shape, and become a prion, too. Mad cow and scrapie affect cows and sheep, respectively, and Creutzfeld-Jakob disease (CJD) affects people.

In the new study, Aguzzi's team used chemicals called luminescent conjugated polythiophenes, which are fluorescent compounds that researchers often use to stain tissue samples, so they can see what's happening in cells. The team first infected mice with a prion disease, then injected them with polythiophenes.

They found that some polythiophenes extended the mice's survival by more than 80 percent, compared with control mice that were not injected with polythiophenes.

This meant some mice survived up to 140 days after infection if they got the polythiophene before being infected with prions, and up to 100 days if they were treated once infected. "I was pretty surprised it worked so well," Aguzzi said.

livescience.com/51763-prion-di…

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