Authors: Natalie Morton, Future Science Group
Researchers from the Case Western Reserve University School of Medicine (CWRSM; OH, USA) have synthesized the first human prion – a huge step towards better understanding exactly how and why these misfolded proteins cause brain disease and deterioration.
Prions are misfolded proteins that interact and bind with other normal proteins in the brain, causing a variety of diseases, known as prion diseases. Creutzfeldt-Jakob disease is the most common of these illnesses, and is characterized by microscopic holes in the brain matter, leading to dementia and death. Human prion diseases account for about 0.01% of deaths worldwide and there is no known cure.
In this study, published in Nature Communications, researchers synthesized a highly destructive prion from genetically modified Escherichia coli. They discovered that it was not the mere presence of prions that caused disease, but the structural mistakes in the C-terminus that dictated the prions’ growth rates and effects on the brain.