Journal Article (Review Article)

DZNE-2020-06347

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png A critical review of the prion hypothesis of human synucleinopathies.

Tamgüney, G. (First author)DZNE* ; Korczyn, A. D.

2018
Springer Heidelberg

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Please use a persistent id in citations: doi:10.1007/s00441-017-2712-y

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.

Keyword(s): Humans (MeSH) ; Models, Biological (MeSH) ; Neurodegenerative Diseases: metabolism (MeSH) ; Neurodegenerative Diseases: pathology (MeSH) ; Prions: metabolism (MeSH) ; alpha-Synuclein: metabolism (MeSH) ; Prions ; alpha-Synuclein

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Contributing Institute(s):

1. Prion and prion-like diseases (AG Tamgüney 2)

Research Program(s):

1. 342 - Disease Mechanisms and Model Systems (POF3-342) (POF3-342)

Appears in the scientific report 2018

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Database coverage:
; BIOSIS Previews ; Clarivate Analytics Master Journal List ; Current Contents - Life Sciences ; Ebsco Academic Search ; IF < 5 ; JCR ; SCOPUS ; Web of Science Core Collection ; Zoological Record

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