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 …

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 …

Endocytosis

S Mukherjee, RN Ghosh… - Physiological …, 1997 - journals.physiology.org
Mammalian cells take up extracellular material by a variety of different mechanisms that are
collectively termed endocytosis. Endocytic mechanisms serve many important cellular …

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 …

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 …

Cell-free formation of protease-resistant prion protein

DA Kocisko, JH Come, SA Priola, B Chesebro… - Nature, 1994 - nature.com
THE infectious agent (or'prion') of the transmissible spongiform encephalopathies (TSEs)
such as scrapie resembles a virus in that it replicates in vivo and has distinct strains1, but it …

[HTML][HTML] The scrapie-associated form of PrP is made from a cell surface precursor that is both protease-and phospholipase-sensitive.

B Caughey, GJ Raymond - Journal of Biological Chemistry, 1991 - Elsevier
A common feature of scrapie and related transmissible spongiform encephalopathies is the
accumulation of an abnormal protease-resistant form of PrP which may be the major …

Congo red and protein aggregation in neurodegenerative diseases

P Frid, SV Anisimov, N Popovic - Brain research reviews, 2007 - Elsevier
Congo red is a commonly used histological dye for amyloid detection. The specificity of this
staining results from Congo red's affinity for binding to fibril proteins enriched in β-sheet …

Evidence for synthesis of scrapie prion proteins in the endocytic pathway.

DR Borchelt, A Taraboulos, SB Prusiner - Journal of Biological Chemistry, 1992 - ASBMB
Infectious scrapie prions are composed largely, if not entirely, of an abnormal isoform of the
prion protein (PrP) which is designated PrPSc. A chromosomal gene encodes both the …