The biological function of the cellular prion protein: an update

MA Wulf, A Senatore, A Aguzzi - BMC biology, 2017 - Springer
The misfolding of the cellular prion protein (PrP C) causes fatal neurodegenerative
diseases. Yet PrP C is highly conserved in mammals, suggesting that it exerts beneficial …

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 …

Neurodegenerative diseases: expanding the prion concept

LC Walker, M Jucker - Annual review of neuroscience, 2015 - annualreviews.org
The prion paradigm has emerged as a unifying molecular principle for the pathogenesis of
many age-related neurodegenerative diseases. This paradigm holds that a fundamental …

A critical appraisal of the pathogenic protein spread hypothesis of neurodegeneration

DM Walsh, DJ Selkoe - Nature Reviews Neuroscience, 2016 - nature.com
There has been an explosion in the number of papers discussing the hypothesis
of'pathogenic spread'in neurodegenerative disease—the idea that abnormal forms of …

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 …

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 …

Prion protein biology

SB Prusiner, MR Scott, SJ DeArmond, FE Cohen - cell, 1998 - cell.com
Prion Protein Biology: Cell Skip to Main Content Advertisement Cell This journal offers authors
two options (open access or subscription) to publish research Submit Log in Register Log in …

Sporadic and familial CJD: classification and characterisation

P Gambetti, Q Kong, W Zou, P Parchi… - British medical …, 2003 - academic.oup.com
Prion diseases are unique transmissible neurodegenerative diseases that have diverse
phenotypes and can be familial, sporadic, or acquired by infection. Recent findings indicate …

Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene

K Basler, B Oesch, M Scott, D Westaway, M Wälchli… - Cell, 1986 - cell.com
PrP 27-30 is the major protein in purified preparations of scrapie agent. An almost complete
PrP cDNA was used to select PrP-related genomic clones from normal hamster DNA. The …