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 …

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 …

Eight prion strains have PrPSc molecules with different conformations

J Safar, H Wille, V Itri, D Groth, H Serban, M Torchia… - Nature medicine, 1998 - nature.com
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 …

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 …

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 …

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 …

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 …

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 …

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 …

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 …