Cited by 372Prion Diseases and Protein MisfoldingAlzheimer's disease research and treatmentsAmyotrophic Lateral Sclerosis Research

The DOI matches its Crossref record; 173 of the 174 references it cites were found in a registry or an index.

Neurodegenerative Diseases: Expanding the Prion Concept

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Walker, Lary C., Jucker, Mathias·2015·Annual Review of Neuroscience

Abstract

The prion paradigm has emerged as a unifying molecular principle for the pathogenesis of many age-related neurodegenerative diseases. This paradigm holds that a fundamental cause of specific disorders is the misfolding and seeded aggregation of certain proteins. The concept arose from the discovery that devastating brain diseases called spongiform encephalopathies are transmissible to new hosts by agents consisting solely of a misfolded protein, now known as the prion protein. Accordingly, “prion” was defined as a “proteinaceous infectious particle.” As the concept has expanded to include other diseases, many of which are not infectious by any conventional definition, the designation of prions as infectious agents has become problematic. We propose to define prions as “proteinaceous nucleating particles” to highlight the molecular action of the agents, lessen unwarranted apprehension about the transmissibility of noninfectious proteopathies, and promote the wider acceptance of this revolutionary paradigm by the biomedical community.

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  1. Baker HF, 1993, Int. J. Exp. Pathol., 74, 441
  2. DeArmond SJ, 1995, Am. J. Pathol., 146, 785
  3. Liberski PP, 2012, Folia Neuropathol., 50, 85

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Metadata

Authors
Walker, L. C., & Jucker, M.
Affiliations
  • Emory University (US)
  • German Center for Neurodegenerative Diseases (DE)
Venue
Annual Review of Neuroscience
Published
2015-07-08
Volume
38
Issue
1
Pages
87-103
ISSN
  • 0147-006X (ISSN-L)
  • 1545-4126
OpenAlex
W2156606243
Cited by
361
References
174
Citations by year
2026: 18 · 2025: 21 · 2024: 24 · 2023: 22 · 2022: 34 · 2021: 26 · 2020: 48 · 2019: 34 · 2018: 39 · 2017: 40 · 2016: 42 · 2015: 13
FWCI
4.33
Type
journal article
Language
en
Open access
Locations
  • Annual Review of Neuroscience (publishedVersion)
  • PubMed (publishedVersion)
  • Europe PMC (PubMed Central) (acceptedVersion)
  • PubMed Central (acceptedVersion)
  • DZNE Pub (submittedVersion)
Funders
  • MetLife Foundation
  • Bundesministerium für Bildung und Forschung (01GI0705)
  • National Institutes of Health (RR000165, AG025688, OD11132, P51OD011132)
  • National Institute on Aging (AG 025688, R21AG040589, P50AG025688, AG040589)
  • National Center for Research Resources (RR000165, P51RR000165)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
  • Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Keywords
neurodegenerative diseases, protein misfolding, seeded aggregation, spongiform encephalopathy, prion protein, proteinopathies, transmissibility, age-related neurodegeneration, pathogenesis
MeSH terms
Animals, Humans, Prions, Neurodegenerative Diseases

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