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Nature. 2016 Nov 10;539(7628):217-226. doi: 10.1038/nature20415.

Mammalian prions and their wider relevance in neurodegenerative diseases.

Author information

1
Medical Research Council Prion Unit, University College London Institute of Neurology, London WC1N 3BG, UK.
2
Department of Neurodegenerative Disease, University College London Institute of Neurology, London WC1N 3BG, UK.

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.

PMID:
27830781
DOI:
10.1038/nature20415
[Indexed for MEDLINE]

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