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Molecular Mechanisms of Prion Pathogenesis
Open accessAbstract
Prion diseases are infectious neurodegenerative diseases occurring in humans and animals with an invariably lethal outcome. One fundamental mechanistic event in prion diseases is the aggregation of aberrantly folded prion protein into large amyloid plaques and fibrous structures associated with neurodegeneration. The cellular prion protein (PrP C) is absolutely required for disease development, and prion knockout mice are not susceptible to prion disease. Prions accumulate not only in the central nervous system but also in lymphoid organs, as shown for new variant and sporadic Creutzfeldt-Jakob patients and for some animals. To date it is largely accepted that prions consist primarily of PrP Sc, a misfolded and aggregated β-sheet-rich isoform of PrP C. However, PrP Sc may or may not be completely congruent with the infectious moiety. Here, we discuss the molecular mechanisms leading to neurodegeneration, the role of the immune system in prion pathogenesis, and the existence of prion strains that appear to have different tropisms and biochemical characteristics.
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Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis
Prasad, A., Bharathi, V., Sivalingam, V., Girdhar, A., & Patel, B. K. (2019) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2019.00025 · Cited by 774
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Metadata
- Authors
- Aguzzi, A., Sigurdson, C., & Heikenwaelder, M.
- Venue
- Annual Review of Pathology: Mechanisms of Disease
- Publisher
- Annual Reviews
- Published
- 2008-02-01
- Volume
- 3
- Issue
- 1
- Pages
- 11-40
- ISSN
- 1553-4006 (ISSN-L)
- 1553-4014
- OpenAlex
- W2154336998
- Cited by
- 351
- References
- 162
- FWCI
- 2.04
- Type
- journal article
- Language
- en
- Open access
- green
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