학술논문

A critical review of the prion hypothesis of human synucleinopathies.
Document Type
Article
Source
Cell & Tissue Research. Jul2018, Vol. 373 Issue 1, p213-220. 8p.
Subject
*PARKINSON'S disease
*LEWY body dementia
*MULTIPLE system atrophy
*SYNUCLEINS
*PRION diseases
Language
ISSN
0302-766X
Abstract
Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA) are neurodegenerative disorders which have been pathologically classified as synucleinopathies, since they are associated with pathognomonic deposits of misfolded alpha-synuclein in cells of the nervous system. Recently PD, DLB, and MSA were also suggested to be prion-like disorders. Much controversy exists regarding this analogy between synucleinopathies and prion diseases. Here, we discuss what characterizes prion diseases and in which way synucleinopathies may be considered prion-like or -unlike. We critically review recent clinical and in vivo evidence from transmission studies to animals in support of or questioning the prion hypothesis of human synucleinopathies. We conclude that, although PD, DLB, and MSA fulfill many criteria of prion-likeness, they also still fail some of these criteria. [ABSTRACT FROM AUTHOR]