Prion diseases are fatal and infectious neurodegenerative disorders and prototypic conformational diseases. They are caused by the structural conversion of the cellular prion protein (PrP(C)) into the pathological PrPSc isoform. Examples are scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease (CWD) in cervids, and Creutzfeldt-Jacob disease (CJD) in humans. The concept that a protein can constitute an infectious agent is now widely accepted. Even more, prion-like features of proteins playing central roles in the pathogenesis of other neurodegenerative diseases were described in recent years. Prions are transmissible within and sometimes between species. This was highlighted by the BSE epidemic that devastated entire economies and resulted in infections of humans and several other species. Whereas BSE was widely contained by an international package of complementary measures, another animal prion disease is currently taking center stage: CWD. Prion research traditionally uses a highly multi-disciplinary approach, from structural biology to field research. This special issue highlights recent developments and discusses progress and remaining challenges. Leading researchers describe new concepts, experimental models, and technologies that drive prion research. Reviews grouped in chapters describe recent advances in the understanding of the infectious unit and the molecular determinants of prion strains and new findings in the cell biology of prions. Experts discuss the modern diagnosis of prion diseases and its influence on the diagnosis of other neurodegenerative disorders. Finally, therapeutic targets and strategies for therapy and prophylaxis are described, although a final cure for these devastating diseases is not yet at the horizon.
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