Abstract
Prions are notorious protein-only infectious agents that cause invariably fatal brain diseases following silent incubation periods that can span a lifetime. These diseases can arise spontaneously, through infection or be inherited. Remarkably, prions are composed of self-propagating assemblies of a misfolded cellular protein that encode information, generate neurotoxicity and evolve and adapt in vivo. Although parallels have been drawn with Alzheimer's disease and other neurodegenerative conditions involving the deposition of assemblies of misfolded proteins in the brain, insights are now being provided into the usefulness and limitations of prion analogies and their aetiological and therapeutic relevance.
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I am grateful to R. Newton for the preparation of figures and to H. Saibil, D. Walsh, J. Wadsworth, G. Jackson and S. Mead for helpful comments on the manuscript.
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J.C. is a director and shareholder of D-Gen Limited, an academic spin-out company working the field of prion-disease diagnosis, decontamination and therapy.
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Collinge, J. Mammalian prions and their wider relevance in neurodegenerative diseases. Nature 539, 217–226 (2016). https://doi.org/10.1038/nature20415
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DOI: https://doi.org/10.1038/nature20415
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