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A cure for mad cow?

Maya E. Shwayder, AFPAugust 6, 2015

This is the first instance of researchers proving that prions, which are toxic to the brain's neurons, can be cured or prevented.

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Neurons (Rebecca Lee/ Creative Commons)
Image: cc-by-nc-nd/Rebecca Lee

A cure for mad cow disease may be around the corner (and down the street a few kilometers and around a few more corners). In a new study published on Wednesday in the American journal "Science Translational Medicine," researchers say they've developed a compound, called polythiophene, that successfully fights misfolded prion clustering in mice.

"The goal of the study was to bring about proof of the principle that you could use rational design to invent anti-prion compounds, and that goal was achieved," the study's lead author Professor Adriano Aguzzi of the University of Zurich told Deutsche Welle.

However, Aguzzi cautioned, this study did not aim to actually develop a drug that could eventually fight neurodegenerative diseases. "That's what industry is for," Aguzzi said. But of course, the goal is eventually to develop a therapy for humans.

Prions are protein structures that, when they become warped or fold in on themselves improperly, can lead to dementia and neurodegeneration. Infectious prions create a chain reaction by triggering normal proteins to refold abnormally and then form long chains that are toxic to the brain's neurons.

Prions are a major factor in the progress of Creutzfeldt-Jakob disease, the human equivalent of mad cow disease.

Aguzzi said they were testing the compound both in prophylactic setting (pre-infection) and therapeutically (post-infection). This marks the first time a drug compound has proven effective in both preventing and curing protein aggregates.

Aguzzi also said that this similar method could eventually be used to develop a therapy for everything from Alzheimer's to Parkinson's disease, all of which involve similar clustering of these types of mutated proteins.

With AFP