TY - JOUR
T1 - The prion hypothesis
T2 - From biological anomaly to basic regulatory mechanism
AU - Tuite, Mick F.
AU - Serio, Tricia R.
N1 - Funding Information:
The research on yeast prions carried out in the Tuite laboratory is supported by funding from the UK Biotechnology and Biological Sciences Research Council and The Wellcome Trust, UK. Work in the Serio laboratory is supported by the US National Institutes of Health National Institute of General Medical Sciences (NIGMS) and the National Science Foundation (ADVANCE) USA. The authors thank members of the Tuite and Serio laboratories for critical reading of this manuscript before submission. The authors also thank S. Saupe, S. Liebman, C. Cullin and K. Stojanovski for providing images used in figure 2. The authors apologize that owing to space constraints they were unable to directly cite all primary studies that have contributed to our understanding of prion biology and its physiological effects.
PY - 2010/12
Y1 - 2010/12
N2 - 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.
AB - 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.
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U2 - 10.1038/nrm3007
DO - 10.1038/nrm3007
M3 - Review article
C2 - 21081963
AN - SCOPUS:78649417132
VL - 11
SP - 823
EP - 833
JO - Nature Reviews Molecular Cell Biology
JF - Nature Reviews Molecular Cell Biology
SN - 1471-0072
IS - 12
ER -