Prion Progress

Although disease-causing prions are an extreme pathogenic aberration in protein behavior, emerging evidence places them at the end of a broad spectrum of ordered protein aggregates with diverse biological roles. This Select highlights recent findings on prions, including mutations that push proteins with prion-like domains over the threshold to pathogenicity in degenerative disorders, a vertebrate model of prion disease with the fastest known incubation time, and a system to examine howcytoplasmic prions are transmitted between cells. A unique therapeutic perspective is also offered by a recent study that suggests potential beneficial effects of prion-like amyloid fibrils in neuroinflammatory diseases, such as multiple sclerosis.