Published in

Nature Research, Nature Reviews Molecular Cell Biology, 12(11), p. 823-833, 2010

DOI: 10.1038/nrm3007

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The prion hypothesis: from biological anomaly to basic regulatory mechanism

Journal article published in 2010 by Mick F. Tuite ORCID, Tricia R. Serio
This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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Abstract

Prions are unusual proteinaceous infectious agents that are typically associated with a class of fatal degenerative diseases of the mammalian brain. However, the discovery of fungal prions, which are not associated with disease, suggests that we must now consider the effect of these factors on basic cellular physiology in a different light. Fungal prions are epigenetic determinants that can alter a range of cellular processes, including metabolism and gene expression pathways, and these changes can lead to a range of prion-associated phenotypes. The mechanistic similarities between prion propagation in mammals and fungi suggest that prions are not a biological anomaly but instead could be a newly appreciated and perhaps ubiquitous regulatory mechanism.