Cited by 285Parkinson's Disease Mechanisms and TreatmentsAlzheimer's disease research and treatmentsNuclear Receptors and Signaling

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The prion hypothesis in Parkinson's disease: Braak to the future

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Visanji, Naomi P, Brooks, Patricia L, Hazrati, Lili-Naz, Lang, Anthony E·2013·Acta Neuropathologica Communications

Abstract

Parkinson's disease (PD) is a progressive neurodegenerative disorder typified by the presence of intraneuronal inclusions containing aggregated alpha synuclein (αsyn). The progression of parkinsonian pathology and clinical phenotype has been broadly demonstrated to follow a specific pattern, most notably described by Braak and colleagues. In more recent times it has been hypothesized that αsyn itself may be a critical factor in mediating transmission of disease pathology from one brain area to another. Here we investigate the growing body of evidence demonstrating the ability of αsyn to spread transcellularly and induce pathological aggregation affecting neurons by permissive templating and provide a critical analysis of some irregularities in the hypothesis that the progression of PD pathology may be mediated by such a prion-like process. Finally we discuss some key questions that remain unanswered which are vital to determining the potential contribution of a prion-like process to the pathogenesis of PD.

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Authors
Visanji, N. P., Brooks, P. L., Hazrati, L.-N., & Lang, A. E.
Affiliations
  • Toronto Western Hospital (CA)
  • University of Toronto (CA)
Corresponding author
Lang, Anthony E
Venue
Acta Neuropathologica Communications
Published
2013-05-08
Volume
1
Issue
1
Pages
2-2
ISSN
2051-5960 (ISSN-L)
OpenAlex
W2159340140
Cited by
278
References
92
Citations by year
2026: 11 · 2025: 20 · 2024: 23 · 2023: 23 · 2022: 27 · 2021: 23 · 2020: 29 · 2019: 22 · 2018: 18 · 2017: 20 · 2016: 21 · 2015: 22 · 2014: 14 · 2013: 5
FWCI
2.69
Type
journal article
Language
en
Open access
Licence
cc-by
Locations
  • Acta Neuropathologica Communications (publishedVersion, cc-by)
  • Europe PMC (PubMed Central) (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • TSpace (University of Toronto) (submittedVersion)
  • eScholarship@McGill (McGill) (submittedVersion, cc-by)
Topics
  • Health Sciences › Medicine › Clinical Neurology › Parkinson's Disease Mechanisms and Treatments
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Nuclear Receptors and Signaling
Keywords
Parkinson's disease, prion hypothesis, alpha-synuclein, prion-like propagation, templating, Braak staging, neurodegeneration, alpha-synuclein aggregation, disease progression
MeSH terms
Animals, Humans, Models, Neurological, Parkinson Disease, Prions, Severity of Illness Index

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