Most infectious diseases are caused by viruses, bacteria or parasites. Prion diseases – including BSE in cows, CJD in humans and scrapie in sheep – are different. Instead, they’re caused by an abnormally-shaped version of a protein found in healthy cells. These ‘bad’ prions convert copies of the healthy protein into the dangerous form that causes disease. But exactly how this conversion happens in a mystery. Now researchers have used a high-powered electron microscope to peer at the structure of faulty and healthy prions. They’ve discovered that abnormal prions form a particular spiralling structure called a beta-solenoid that acts as a template for converting healthy prions into the wrong shape, shown in this computer representation with the external view on top, and the internal spiral structure below. This is different to previous ideas about how prions work, and could spark new avenues of research aimed at finding treatments for these incurable diseases.
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