May 16 2024Boston University School of Medicine Prion disease is an umbrella term for a group of fatal and currently untreatable neurodegenerative diseases that not only affect humans, but also wild and captive animals. These diseases include Creutzfeldt-Jakob disease in humans, bovine spongiform encephalopathy , and chronic wasting disease affecting deer, elk and moose.
In a new study, researchers from Boston University Chobanian & Avedisian School of Medicine have identified 10 compounds that are able to reduce PrPSc levels in infected cells and have shown that the most potent molecules can also prevent the toxicity that was observed when applying PrPSc to cultured neurons.
The researchers had initially explored the anti-prion properties of these molecules because they were known to bind to the sigma receptors , which they had reason to believe were involved in prion proliferation. Using gene knockout technology , they determined that the sigma receptors were not the relevant targets of these drugs, from the perspective of their anti-prion properties.
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