Prions
SB Prusiner - Proceedings of the National Academy of …, 1998 - National Acad Sciences
Prions are unprecedented infectious pathogens that cause a group of invariably fatal
neurodegenerative diseases by an entirely novel mechanism. Prion diseases may present …
neurodegenerative diseases by an entirely novel mechanism. Prion diseases may present …
The priori diseases
SB Prusiner - Brain pathology, 1998 - Wiley Online Library
The human prion diseases are fatal neurodegenerative maladies that may present as
sporadic, genetic, or infectious illnesses. The sporadic form is called Creutzfeldt‐Jakob …
sporadic, genetic, or infectious illnesses. The sporadic form is called Creutzfeldt‐Jakob …
Eight prion strains have PrPSc molecules with different conformations
Variations in prions, which cause different incubation times and deposition patterns of the
prion protein isoform called PrP Sc, are often referred to as' strains'. We report here a highly …
prion protein isoform called PrP Sc, are often referred to as' strains'. We report here a highly …
Analysis of 27 mammalian and 9 avian PrPs reveals high conservation of flexible regions of the prion protein
F Wopfner, G Weidenhöfer, R Schneider… - Journal of molecular …, 1999 - Elsevier
Prion diseases are fatal neurodegenerative disorders in man and animal associated with
conformational conversion of a cellular prion protein (PrPc) into the pathologic isoform …
conformational conversion of a cellular prion protein (PrPc) into the pathologic isoform …
Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans
MR Scott, R Will, J Ironside… - Proceedings of the …, 1999 - National Acad Sciences
There is growing concern that bovine spongiform encephalopathy (BSE) may have passed
from cattle to humans. We report here that transgenic (Tg) mice expressing bovine (Bo) prion …
from cattle to humans. We report here that transgenic (Tg) mice expressing bovine (Bo) prion …
EEG in Creutzfeldt–Jakob disease
HG Wieser, K Schindler, D Zumsteg - Clinical Neurophysiology, 2006 - Elsevier
Electroecenphalography (EEG) is an integral part of the diagnostic process in patients with
Creutzfeldt–Jakob disease (CJD). The EEG has therefore been included in the World Health …
Creutzfeldt–Jakob disease (CJD). The EEG has therefore been included in the World Health …
The cellular prion protein mediates neurotoxic signalling of β‐sheet‐rich conformers independent of prion replication
UK Resenberger, A Harmeier, AC Woerner… - The EMBO …, 2011 - embopress.org
Formation of aberrant protein conformers is a common pathological denominator of different
neurodegenerative disorders, such as Alzheimer's disease or prion diseases. Moreover …
neurodegenerative disorders, such as Alzheimer's disease or prion diseases. Moreover …
The state of the prion
C Weissmann - Nature Reviews Microbiology, 2004 - nature.com
There is little doubt that the main component of the transmissible agent of spongiform
encephalopathies—the prion—is a conformational variant of the ubiquitous host protein …
encephalopathies—the prion—is a conformational variant of the ubiquitous host protein …
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 …
Animal prion diseases: the risks to human health
F Houston, O Andréoletti - Brain pathology, 2019 - Wiley Online Library
Transmissible spongiform encephalopathies (TSEs) or prion diseases of animals notably
include scrapie in small ruminants, chronic wasting disease (CWD) in cervids and classical …
include scrapie in small ruminants, chronic wasting disease (CWD) in cervids and classical …