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 …

Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

H Ilieva, M Polymenidou, DW Cleveland - Journal of Cell Biology, 2009 - rupress.org
Selective degeneration and death of one or more classes of neurons is the defining feature
of human neurodegenerative disease. Although traditionally viewed as diseases mainly …

Prion diseases

MD Geschwind - CONTINUUM: Lifelong Learning in Neurology, 2015 - journals.lww.com
Abstract Purpose of Review: This article presents an update on the clinical aspects of human
prion disease, including the wide spectrum of their presentations. Recent Findings: Prion …

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 …

Scrapie prion protein contains a phosphatidylinositol glycolipid

N Stahl, DR Borchelt, K Hsiao, SB Prusiner - Cell, 1987 - cell.com
The scrapie (PrPsC) and cellular (PrPc) prion proteins are encoded by the same gene, and
their different properties am thought to arise from posttranslational modifications. We have …

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 …

[HTML][HTML] Physiological functions of the cellular prion protein

AR Castle, AC Gill - Frontiers in molecular biosciences, 2017 - frontiersin.org
The prion protein, PrPC, is a small, cell-surface glycoprotein notable primarily for its critical
role in pathogenesis of the neurodegenerative disorders known as prion diseases. A …

[HTML][HTML] Prion protein biology

SB Prusiner, MR Scott, SJ DeArmond, FE Cohen - cell, 1998 - cell.com
Prion Protein Biology: Cell Skip to Main Content Review| Volume 93, ISSUE 3, P337-348, May
01, 1998 PDF [871 KB]PDF [871 KB] Save Add to Online LibraryPowered ByMendeley Add to …