[Is Parkinson's disease a prion disease?].

Abstract

The accumulation of a specific protein in aggregated form is a common phenomenon in human neurodegenerative diseases. In Parkinson's disease, this protein is α-synuclein which is a neuronal protein of 143 amino acids. With a monomeric conformation in solution, it also has a natural capacity to aggregate into amyloid structures (dimers, oligomers, fibrils and Lewy bodies or neurites). It therefore fulfils the characteristics of a prion protein (different conformations, seeding and spreading). In vitro and in vivo experimental evidence in transgenic and wild animals indicates a prion-like propagation of Parkinson's disease. The sequential and predictive distribution of α-synuclein demonstrated by Braak et al. and its correlation with non-motor signs are consistent with the prion-like progression. Although the triggering factor causing the misfolding and aggregation of the target protein is unknown, Parkinson's disease is a highly relevant model for the study of these mechanisms and also to test specific treatments targeting the assemblies of α-synuclein and propagation from pre-motor phase of the disease. Despite this prion-like progression, there is currently no argument indicating a risk of human transmission of Parkinson's disease.

• Authors: J-P Brandel, A-G Corbillé, P Derkinderen, S Haïk
• Journal: Revue neurologique (Rev Neurol (Paris)) Vol. 171 Issue 12 Pg. 812-24 (Dec 2015) ISSN: 0035-3787 [Print] France
• Vernacular Title: La maladie de Parkinson est-elle une maladie à prion?
• PMID: 26563663 (Publication Type: English Abstract, Journal Article, Review)
• Copyright: Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Chemical References

• Prions
• Synucleins

Topics

• Humans
• Parkinson Disease (etiology)
• Prion Diseases
• Prions (metabolism)
• Synucleins (metabolism)