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 …

Molecular biology of prion diseases

SB Prusiner - Science, 1991 - science.org
Prions cause transmissible and genetic neurodegenerative diseases, including scrapie and
bovine spongiform encephalopathy of animals and Creutzfeldt-Jakob and Gerstmann …

Prions

DW Colby, SB Prusiner - Cold Spring Harbor …, 2011 - cshperspectives.cshlp.org
The discovery of infectious proteins, denoted prions, was unexpected. After much debate
over the chemical basis of heredity, resolution of this issue began with the discovery that …

Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein

H Büeler, M Fischer, Y Lang, H Bluethmann, HP Lipp… - Nature, 1992 - nature.com
PrPc is a host protein anchored to the outer surface of neurons and to a lesser extent of
lymphocytes and other cells. The transmissible agent (prion) responsible for scrapie is …

Transmissions to mice indicate that 'new variant'CJD is caused by the BSE agent

ME Bruce, RG Will, JW Ironside, I McConnell… - Nature, 1997 - nature.com
There are many strains of the agents that cause transmissible spongiform encephalopathies
(TSEs) or 'prion'diseases. These strains are distinguishable by their disease characteristics …

Prions: protein aggregation and infectious diseases

A Aguzzi, AM Calella - Physiological reviews, 2009 - journals.physiology.org
Transmissible spongiform encephalopathies (TSEs) are inevitably lethal neurodegenerative
diseases that affect humans and a large variety of animals. The infectious agent responsible …

Physiology of the prion protein

R Linden, VR Martins, MAM Prado… - Physiological …, 2008 - journals.physiology.org
Prion diseases are transmissible spongiform encephalopathies (TSEs), attributed to
conformational conversion of the cellular prion protein (PrPC) into an abnormal conformer …

Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication

SB Prusiner, M Scott, D Foster, KM Pan, D Groth… - Cell, 1990 - cell.com
Transgenic (Tg) mice expressing both Syrian hamster (Ha) and mouse (Mo) prion protein
(PrP) genes were used to probe the mechanism of scrapie prion replication. Four Tg lines …

Prion diseases and the BSE crisis

SB Prusiner - Science, 1997 - science.org
Bovine spongiform encephalopathy (BSE) and human Creutzfeldt-Jakob disease (CJD) are
among the most notable central nervous system degenerative disorders caused by prions …

Secondary structure analysis of the scrapie-associated protein PrP 27-30 in water by infrared spectroscopy

BW Caughey, A Dong, KS Bhat, D Ernst, SF Hayes… - Biochemistry, 1991 - ACS Publications
Byron W. Caughey,*'* Aichun Dong,* Kolari S. Bhat,* Darwin Ernst,* Stanley F. Hayes,* and
Winslow S. Caughey* NIAID, NationalInstitutes of Health, Rocky Mountain Laboratories …