Mad cows and psi-chotic yeast: an extension of the prion hypothesis

1999 ◽  
Vol 35 (3-4) ◽  
pp. 150-150
Author(s):  
S. Lindquist ◽  
T. Serio ◽  
A. Cashikar ◽  
J. Glover ◽  
A. Kowal ◽  
...  
Keyword(s):  
Prion Hypothesis

Prion hypothesis proved?

2005 ◽  
Vol 4 (6) ◽  
pp. 337
Author(s):  
Helen Pilcher
Keyword(s):  
Prion Hypothesis

scholarly journals Human tauopathy-derived tau strains determine the substrates recruited for templated amplification

Brain ◽  
2021 ◽  
Author(s):  
Airi Tarutani ◽  
Haruka Miyata ◽  
Takashi Nonaka ◽  
Kazuko Hasegawa ◽  
Mari Yoshida ◽  
...  
Keyword(s):  
Cultured Cells ◽  
Corticobasal Degeneration ◽  
Biochemical Properties ◽  
Cellular Model ◽  
Cryo Electron Microscopy ◽  
Structural Differences ◽  
The Brain ◽  
Strain Dependent ◽  
Disease Specific ◽  
Prion Hypothesis

Abstract Tauopathies are a subset of neurodegenerative diseases characterized by abnormal tau inclusions. Specifically, three-repeat tau and four-repeat tau in Alzheimer’s disease (AD), three-repeat tau in Pick's disease (PiD) and four-repeat in progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) form amyloid-like fibrous structures that accumulate in neurons and/or glial cells. Amplification and cell-to-cell transmission of abnormal tau based on the prion hypothesis are believed to explain the onset and progression of tauopathies. Recent studies support not only the self-propagation of abnormal tau, but also the presence of conformationally distinct tau aggregates, namely tau strains. Cryo-electron microscopy analyses of patient-derived tau filaments have revealed disease-specific ordered tau structures. However, it remains unclear whether the ultrastructural and biochemical properties of tau strains are inherited during the amplification of abnormal tau in the brain. In this study, we investigated template-dependent amplification of tau aggregates using a cellular model of seeded aggregation. Tau strains extracted from human tauopathies caused strain-dependent accumulation of insoluble filamentous tau in SH-SY5Y cells. The seeding activity towards full-length four-repeat tau substrate was highest in CBD-tau seeds, followed by PSP-tau and AD-tau seeds, while AD-tau seeds showed higher seeding activity than PiD-tau seeds towards three-repeat tau substrates. Abnormal tau amplified in cells inherited the ultrastructural and biochemical properties of the original seeds. These results strongly suggest that the structural differences of patient-derived tau strains underlie the diversity of tauopathies, and that seeded aggregation and filament formation mimicking the pathogenesis of sporadic tauopathy can be reproduced in cultured cells. Our results indicate that the disease-specific conformation of tau aggregates determines the tau isoform substrate that is recruited for templated amplification, and also influences the prion-like seeding activity.

scholarly journals Mad Cows Meet Psi-chotic Yeast: The Expansion of the Prion Hypothesis

Cell ◽  
1997 ◽  
Vol 89 (4) ◽  
pp. 495-498 ◽  
Author(s):  
Susan Lindquist
Keyword(s):  
Prion Hypothesis

The scrapie agent and the prion hypothesis

1988 ◽  
Vol 13 (8) ◽  
pp. 309-313 ◽  
Author(s):  
Maurizio Brunori ◽  
Maria Chiara Silvestrini ◽  
Maurizio Pocchiari
Keyword(s):  
Scrapie Agent ◽  
Prion Hypothesis

Straining the prion hypothesis

Nature ◽  
10.1038/34818 ◽  
1998 ◽  
Vol 391 (6665) ◽  
pp. 345-346 ◽  
Author(s):  
Christine F. Farquhar ◽  
Robert A. Somerville ◽  
Moira E. Bruce
Keyword(s):  
Prion Hypothesis
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