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Publication

ɑ-Synuclein Strains and Seeding in Parkinson’s Disease, Incidental Lewy Body Disease, Dementia with Lewy Bodies and Multiple System Atrophy: Similarities and Differences

Journal Contribution - Journal Article

Several age-related neurodegenerative disorders are characterized by the deposition of aberrantly folded endogenous proteins. These proteins have prion-like propagation and amplification properties but so far appear nontransmissible between individuals. Because of the features they share with the prion protein, PrP, the characteristics of pathogenic protein aggregates in several progressive brain disorders, including different types of Lewy body diseases (LBDs), such as Parkinson's disease (PD), multiple system atrophy (MSA) and dementia with Lewy bodies (DLB), have been actively investigated. Even though the pleomorphic nature of these syndromes might suggest different underlying causes, ɑ-synuclein (ɑSyn) appears to play an important role in this heterogeneous group of diseases (the synucleinopathies). An attractive hypothesis is that different types of ɑSyn protein assemblies have a unique and causative role in distinct synucleinopathies. We will discuss the recent research progress on ɑSyn assemblies involved in PD, MSA and DLB; their behavior as strains; current spreading hypotheses; their ability to seed centrally and peripherally; and their implication for disease pathogenesis.
Journal: Cell and Tissue Research
ISSN: 0302-766X
Issue: 1
Volume: 373
Pages: 195 - 212
Publication year:2018
BOF-keylabel:yes
IOF-keylabel:yes
BOF-publication weight:1
CSS-citation score:2
Authors:International
Authors from:Higher Education
Accessibility:Closed