Emerging principles of conformation-based prion inheritance
P Chien, JS Weissman… - Annual review of …, 2004 - annualreviews.org
▪ Abstract The prion hypothesis proposes that proteins can act as infectious agents.
Originally formulated to explain transmissible spongiform encephalopathies (TSEs), the …
Originally formulated to explain transmissible spongiform encephalopathies (TSEs), the …
Prion diseases and their biochemical mechanisms
NJ Cobb, WK Surewicz - Biochemistry, 2009 - ACS Publications
Prion diseases, also known as the transmissible spongiform encephalopathies (TSEs), are a
group of fatal neurodegenerative disorders that affect humans and animals. These diseases …
group of fatal neurodegenerative disorders that affect humans and animals. These diseases …
[HTML][HTML] The prion strain phenomenon: molecular basis and unprecedented features
Prions are unconventional infectious agents responsible for transmissible spongiform
encephalopathies. Compelling evidences indicate that prions are composed exclusively by …
encephalopathies. Compelling evidences indicate that prions are composed exclusively by …
Prion agent diversity and species barrier
V Béringue, JL Vilotte, H Laude - Veterinary research, 2008 - hal.science
Mammalian prions are the infectious agents responsible for transmissible spongiform
encephalopathies (TSE), a group of fatal, neurodegenerative diseases, affecting both …
encephalopathies (TSE), a group of fatal, neurodegenerative diseases, affecting both …
A change in the conformation of prions accompanies the emergence of a new prion strain
D Peretz, RA Williamson, G Legname, Y Matsunaga… - Neuron, 2002 - cell.com
To investigate the role of the pathogenic prion protein (PrP Sc) in controlling susceptibility to
foreign prions, two Syrian hamster (SHa) prion strains, Sc237 and DY, were transmitted to …
foreign prions, two Syrian hamster (SHa) prion strains, Sc237 and DY, were transmitted to …
De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis
CJ Sigurdson, KPR Nilsson… - Proceedings of the …, 2009 - National Acad Sciences
Most transmissible spongiform encephalopathies arise either spontaneously or by infection.
Mutations of PRNP, which encodes the prion protein, PrP, segregate with phenotypically …
Mutations of PRNP, which encodes the prion protein, PrP, segregate with phenotypically …
Proteostatic imbalance and protein spreading in amyotrophic lateral sclerosis
ME Cicardi, L Marrone, M Azzouz, D Trotti - The EMBO journal, 2021 - embopress.org
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder whose exact
causative mechanisms are still under intense investigation. Several lines of evidence …
causative mechanisms are still under intense investigation. Several lines of evidence …
The prion hypothesis: from biological anomaly to basic regulatory mechanism
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 …
of fatal degenerative diseases of the mammalian brain. However, the discovery of fungal …
A prion disease of cervids: chronic wasting disease
C Sigurdson - Veterinary research, 2008 - hal.science
Chronic wasting disease (CWD) is a prion disease of deer, elk, and moose, initially
recognized in Colorado mule deer. The discovery of CWD beyond the borders of Colorado …
recognized in Colorado mule deer. The discovery of CWD beyond the borders of Colorado …
Prion-like proteins in phase separation and their link to disease
ML Sprunger, ME Jackrel - Biomolecules, 2021 - mdpi.com
Aberrant protein folding underpins many neurodegenerative diseases as well as certain
myopathies and cancers. Protein misfolding can be driven by the presence of distinctive …
myopathies and cancers. Protein misfolding can be driven by the presence of distinctive …