Researchers discover critical step in formation of fatal brain diseases

A research team from Imperial College London and the University of Zurich have identified what causes normal proteins to convert to diseased form, causing conditions like Creutzfeldt-Jakob disease (CJD) and Kuru. The researchers also tested a way to block the process, which could lead to new drugs for combatting such diseases.

Certain brain diseases are caused by proteins called prions that malfunction and “misfold”, developing into a form that can accumulate and kill brain cells. These diseases can take decades to manifest but become fatal.

In a paper published in Proceedings of the National Academy of Sciences, researchers have determined the mechanism that turns normal prions into their pathogenic form, which has previously been unknown. The research was supported by Alzheimer’s Research UK.

Lead researcher Professor Alfonso De Simone, from the Department of Life Sciences at Imperial, said: “Prion diseases are aggressive and devastating, and currently there is no cure. Discovering the mechanism by which prions become pathogenic is a crucial step in one day tackling these diseases, as it allows us to search for new drugs. Now we know what we’re targeting, we know what features drugs need to have to stop prions becoming pathogenic.”

While requiring further development for use, the study shows it is possible to disrupt the mechanism, allowing researchers to move forward.

Dr Rosa Sancho, Head of Research at Alzheimer’s Research UK, said: “As the UK’s leading dementia research charity, we are pleased to fund this sophisticated work using biophysical and computational approaches to better understand the role fragments like this play in the development of disease. To identify new ways to reduce or combat these protein fragments in human disease we need to see sustained investment in dementia research.”

Image credit: Morgan Housel

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