A critical review of the prion hypothesis of human synucleinopathies

Gültekin Tamgüney*, Amos D. Korczyn

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review


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 prionlike 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.

Original languageEnglish
Pages (from-to)213-220
Number of pages8
JournalCell and Tissue Research
Issue number1
StatePublished - 8 Nov 2018


  • Creutzfeldt-Jakob disease
  • Parkinson’s disease
  • Prion
  • Prion-like
  • α-Synuclein


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