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Calling α-synuclein a prion is scientifically justifiable

  • Joel C. WattsEmail author
Commentary Letter

For more than a decade, the “prion-like” hypothesis for Parkinson’s disease (PD) has remained one of the most controversial topics in neurodegenerative disease research [21, 34]. Seminal studies revealed that α-synuclein pathology in the brains of PD patients follows a stereotypical progression pattern and that α-synuclein aggregates can spread between cells [3, 9], leading to the theory that a cell-to-cell propagation of protein misfolding may drive disease progression in PD and related synucleinopathies such as dementia with Lewy bodies and multiple system atrophy (MSA). At the core of the prion-like hypothesis is the notion that self-propagating α-synuclein aggregates are able to escape from a cell, enter a neighboring cell, and then act as a seed to induce the aggregation of α-synuclein in the recipient cell. This non-cell autonomous mechanism is similar to what occurs in prion diseases such as Creutzfeldt-Jakob disease (CJD), where misfolded prion protein (PrP) catalyzes the...

Notes

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Tanz Centre for Research in Neurodegenerative Diseases, Department of BiochemistryUniversity of TorontoTorontoCanada

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