Corrupted proteins in focus: How shape gives rise to variations of fatal brain disease: Researchers believe imaging could lead to better understanding of what causes fatal prion diseases - Science Daily
Prion diseases are incurable, deadly neurological disorders that can affect both humans and animals-including Creutzfeldt-Jakob Disease (CJD) in people, Bovine Spongiform Encephalopathy (also known as Mad Cow Disease) and Chronic Wasting Disease found in deer. Scientists have spent decades working to better understand how prions cause these diseases. But new research from the Case Western Reserve University School of Medicine and the National Institutes of Health (NIH) Rocky Mountain Laboratories brings into focus how prions might be formed and how they can result in different disease outcomes. This collaborative research team discovered a new prion structure through revolutionary new imaging using specialized electron microscopes at the Cleveland Center for Structural and Membrane Biology Cryo-Electron Microscopy Core and the Rocky Mountain Laboratories. The new structure is of a second prion strain, providing an atomic-level view into how corrupted prion proteins self-convert and