Key Points
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There are six fungal prions: four are self-propagating amyloids and two are self-activating enzymes.
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[URE3] is a prion of the nitrogen catabolism regulator Ure2p; [PSI+] is a prion of the translation-termination factor Sup35p; [PIN+] is a prion of Rnq1p (function unknown); [Het-s] is a prion of the heterokaryon incompatibility protein HETs; [β] is a prion of vacuolar protease B; and [C] is a prion of a mitogen-activated protein kinase kinase kinase.
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The infectious amyloid of Sup35p has a parallel in-register β-sheet structure.
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The [Het-s] prion of Podospora anserina apparently benefits its host, but the [URE3] and [PSI+] prions of Saccharomyces cerevisiae are detrimental.
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Chaperones catalyse amyloid filament breakage to form new seeds, and probably have other roles in prion propagation and generation as well.
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Different prion variants, with the same protein sequence, have different amyloid structures. Variants can determine host range and chaperone effects.
Abstract
The term 'prion' means an infectious protein that does not need an accompanying nucleic acid. There are six fungal prions, including four self-propagating amyloids and two enzymes that are necessary to activate their inactive precursors. Here we explore the scope of the prion phenomenon, the biological and evolutionary roles of prions, the structural basis of the amyloid prions and the prominent role of chaperones (proteins that affect the folding of other proteins) and other cellular components in prion generation and propagation.
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This research was supported by the Intramural Research Program of the National Institutes of Health, The National Institute of Diabetes and Digestive and Kidney Diseases.
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DATABASES
Entrez Genome Project
Entrez Protein
Glossary
- Prion
-
An infectious protein that does not require a nucleic acid for infectivity.
- Amyloid
-
A filamentous form of protein with a cross β-sheet structure, meaning that the β-strands are perpendicular to the long axis of the filaments.
- Non-chromosomal (cytoplasmic) genetic element
-
A gene or replicon that is inherited or transmitted independently of the chromosomes, such as the mitochondrial genome, the 2 μm plasmid, a yeast virus or a prion.
- Gene gun
-
A device that uses a pneumatic gun to propel gold particles coated with DNA or protein into cells to genetically transform them.
- Prion seed
-
An amyloid fragment that can grow, become fragmented again and thus propagate the prion. Similarly, active enzyme molecules of the [β] and [C] prions can act as seeds.
- Parallel in-register β-sheet
-
A β-sheet in which each residue is aligned with the same residue of the adjacent strand.
- Nuclear magnetic resonance
-
(NMR). A technique in which the distances between labelled nuclei can be measured by the rate of decay of the magnetic signal due to dipole–dipole coupling.
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Wickner, R., Edskes, H., Shewmaker, F. et al. Prions of fungi: inherited structures and biological roles. Nat Rev Microbiol 5, 611–618 (2007). https://doi.org/10.1038/nrmicro1708
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DOI: https://doi.org/10.1038/nrmicro1708
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