Alzheimer's and Parkinson's diseases: The prion concept in relation to assembled Aβ, tau, and α-synuclein

M Goedert - Science, 2015 - science.org
BACKGROUND Alzheimer's disease (AD) and Parkinson's disease (PD) are the most
common human neurodegenerative diseases. AD is primarily a dementing disease, and PD …

Prions, prionoids and protein misfolding disorders

C Scheckel, A Aguzzi - Nature Reviews Genetics, 2018 - nature.com
Prion diseases are progressive, incurable and fatal neurodegenerative conditions. The term
'prion'was first nominated to express the revolutionary concept that a protein could be …

Mutation in the α-synuclein gene identified in families with Parkinson's disease

MH Polymeropoulos, C Lavedan, E Leroy, SE Ide… - science, 1997 - science.org
Parkinson's disease (PD) is a common neurodegenerative disorder with a lifetime incidence
of approximately 2 percent. A pattern of familial aggregation has been documented for the …

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 …

Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins.

KM Pan, M Baldwin, J Nguyen… - Proceedings of the …, 1993 - National Acad Sciences
Prions are composed largely, if not entirely, of prion protein (PrPSc in the case of scrapie).
Although the formation of PrPSc from the cellular prion protein (PrPC) is a post-translational …

Unfolding the role of protein misfolding in neurodegenerative diseases

C Soto - Nature Reviews Neuroscience, 2003 - nature.com
Recent evidence indicates that diverse neurodegenerative diseases might have a common
cause and pathological mechanism—the misfolding, aggregation and accumulation of …

Prion-like mechanisms in neurodegenerative diseases

B Frost, MI Diamond - Nature Reviews Neuroscience, 2010 - nature.com
Many non-infectious neurodegenerative diseases are associated with the accumulation of
fibrillar proteins. These diseases all exhibit features that are reminiscent of those 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 …

Mice devoid of PrP are resistant to scrapie

H Büeler, A Aguzzi, A Sailer, RA Greiner, P Autenried… - Cell, 1993 - Elsevier
SB Prusiner proposed that the infectious agent of scrapie, the prion, is PrP Sc, a modified
form of the normal host protein PrP c. Prn-p 0/0 mice devoid of PrP c showed normal …

Biology and genetics of prions causing neurodegeneration

SB Prusiner - Annual review of genetics, 2013 - annualreviews.org
Prions are proteins that acquire alternative conformations that become self-propagating.
Transformation of proteins into prions is generally accompanied by an increase in β-sheet …