TY  - JOUR
AU  - Tamgüney, Gültekin
AU  - Korczyn, Amos D
TI  - A critical review of the prion hypothesis of human synucleinopathies.
JO  - Cell & tissue research
VL  - 373
IS  - 1
SN  - 0302-766X
CY  - Heidelberg
PB  - Springer
M1  - DZNE-2020-06347
SP  - 213-220
PY  - 2018
AB  - 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.
KW  - Humans
KW  - Models, Biological
KW  - Neurodegenerative Diseases: metabolism
KW  - Neurodegenerative Diseases: pathology
KW  - Prions: metabolism
KW  - alpha-Synuclein: metabolism
KW  - Prions (NLM Chemicals)
KW  - alpha-Synuclein (NLM Chemicals)
LB  - PUB:(DE-HGF)16
C6  - pmid:29116402
DO  - DOI:10.1007/s00441-017-2712-y
UR  - https://pub.dzne.de/record/140025
ER  -