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 …

Oxidative stress and the prion protein in transmissible spongiform encephalopathies

O Milhavet, S Lehmann - Brain research reviews, 2002 - Elsevier
Transmissible spongiform encephalopathies form a group of fatal neurodegenerative
disorders that have the unique property of being infectious, sporadic or genetic in origin …

Scrapie prion protein accumulation by scrapie-infected neuroblastoma cells abrogated by exposure to a prion protein antibody

M Enari, E Flechsig… - Proceedings of the …, 2001 - National Acad Sciences
Exposure of susceptible neuroblastoma N2a cells to mouse scrapie prions leads to infection,
as evidenced by the continued presence of the scrapie form of the prion protein (PrPSc) and …

A quantitative, highly sensitive cell-based infectivity assay for mouse scrapie prions

PC Klöhn, L Stoltze, E Flechsig… - Proceedings of the …, 2003 - National Acad Sciences
Prions are usually quantified by bioassays based on intracerebral inoculation of mice that
are slow, imprecise, and costly. We have isolated neuroblastoma N2a sublines highly …

Screening of 145 anti-PrP monoclonal antibodies for their capacity to inhibit PrPSc replication in infected cells

C Féraudet, N Morel, S Simon, H Volland… - Journal of Biological …, 2005 - ASBMB
Prion diseases are transmissible neurodegenerative disorders affecting humans and
animals for which no therapeutic or prophylactic regimens exist. During the last three years …

New inhibitors of scrapie-associated prion protein formation in a library of 2,000 drugs and natural products

DA Kocisko, GS Baron, R Rubenstein, J Chen… - Journal of …, 2003 - Am Soc Microbiol
Transmissible spongiform encephalopathies (TSEs) are fatal, untreatable
neurodegenerative diseases associated with the accumulation of a disease-specific form of …

Prion strain discrimination in cell culture: the cell panel assay

SP Mahal, CA Baker, CA Demczyk… - Proceedings of the …, 2007 - National Acad Sciences
Prions are thought to consist mainly or entirely of misfolded PrP, a constitutively expressed
host protein. Prions associated with the same PrP sequence may occur in the form of …

Evaluation of quinacrine treatment for prion diseases

A Barret, F Tagliavini, G Forloni, C Bate… - Journal of …, 2003 - Am Soc Microbiol
Based on in vitro observations in scrapie-infected neuroblastoma cells, quinacrine has
recently been proposed as a treatment for Creutzfeldt-Jakob disease (CJD), including a new …

Ex vivo propagation of infectious sheep scrapie agent in heterologous epithelial cells expressing ovine prion protein

D Vilette, O Andréoletti, F Archer… - Proceedings of the …, 2001 - National Acad Sciences
Transmissible spongiform encephalopathies, or prion diseases, are fatal degenerative
disorders of the central nervous system that affect humans and animals. Prions are …

Cationic phosphorus-containing dendrimers reduce prion replication both in cell culture and in mice infected with scrapie

J Solassol, C Crozet, V Perrier… - Journal of General …, 2004 - microbiologyresearch.org
Over the last 30 years, many drugs have been tested both in cell culture and in vivo for their
ability to prevent the generation of prions and the development of transmissible spongiform …