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000140025 0247_ $$2ISSN$$a0373-031X
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000140025 037__ $$aDZNE-2020-06347
000140025 041__ $$aEnglish
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000140025 1001_ $$0P:(DE-2719)2810376$$aTamgüney, Gültekin$$b0$$eFirst author$$udzne
000140025 245__ $$aA critical review of the prion hypothesis of human synucleinopathies.
000140025 260__ $$aHeidelberg$$bSpringer$$c2018
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000140025 520__ $$aParkinson'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.
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000140025 650_7 $$2NLM Chemicals$$aPrions
000140025 650_7 $$2NLM Chemicals$$aalpha-Synuclein
000140025 650_2 $$2MeSH$$aHumans
000140025 650_2 $$2MeSH$$aModels, Biological
000140025 650_2 $$2MeSH$$aNeurodegenerative Diseases: metabolism
000140025 650_2 $$2MeSH$$aNeurodegenerative Diseases: pathology
000140025 650_2 $$2MeSH$$aPrions: metabolism
000140025 650_2 $$2MeSH$$aalpha-Synuclein: metabolism
000140025 7001_ $$0P:(DE-HGF)0$$aKorczyn, Amos D$$b1
000140025 77318 $$2Crossref$$3journal-article$$a10.1007/s00441-017-2712-y$$b : Springer Science and Business Media LLC, 2017-11-08$$n1$$p213-220$$tCell and Tissue Research$$v373$$x0302-766X$$y2017
000140025 773__ $$0PERI:(DE-600)1458496-7$$a10.1007/s00441-017-2712-y$$gVol. 373, no. 1, p. 213 - 220$$n1$$p213-220$$q373:1<213 - 220$$tCell & tissue research$$v373$$x0302-766X$$y2018
000140025 8564_ $$uhttps://pub.dzne.de/record/140025/files/DZNE-2020-06347_Restricted.pdf
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000140025 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2810376$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b0$$kDZNE
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000140025 9141_ $$y2018
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000140025 9201_ $$0I:(DE-2719)1013022$$kAG Tamgüney 2$$lPrion and prion-like diseases$$x0
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