Related Experiment Video
Updated: Feb 27, 2026
12:57
Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
Published on: January 8, 2015
16.6K
Aβ seeds and prions: How close the fit?
Jay Rasmussen1,2,3, Mathias Jucker1,2, Lary C Walker4
1a Department of Cellular Neurology , Hertie Institute for Clinical Brain Research, University of Tübingen , Tübingen , Germany.
Prion
|June 29, 2017
Summary
The prion paradigm explains neurodegenerative diseases. Misfolded amyloid-beta (Aβ) in Alzheimer's disease shares prion-like characteristics, suggesting a unified approach to studying these disorders.
Area of Science:
- Neuroscience
- Molecular Biology
- Pathology
Background:
- The prion paradigm is increasingly used to understand neurodegenerative diseases caused by protein misfolding.
- Misfolded and aggregated amyloid-beta (Aβ) is implicated as the trigger for Alzheimer's disease.
Purpose of the Study:
- To evaluate if Aβ exhibits prion-like characteristics.
- To explore the potential of an expanded prion paradigm to unify research on diverse neurodegenerative disorders.
Main Methods:
- Review of in vitro and in vivo studies on Aβ aggregation.
- Comparison of Aβ characteristics with canonical mammalian prions.
Main Results:
- Aβ exhibits key prion-like features: β-sheet structure, polymerization into amyloid, templated misfolding, strain variability, neuronal spread, and resistance to inactivation.
- Research indicates prion-like protein transformation drives various clinicopathologically diverse diseases.
Conclusions:
- The Aβ peptide in Alzheimer's disease aligns with the prion paradigm.
- An expanded prion paradigm offers a unifying framework for investigating and treating a range of neurodegenerative diseases.
Related Concept Videos
03:03Amyloid Fibrils
12.2K
Amyloid fibrils are aggregates of misfolded proteins. Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils.
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
12.2K
03:03Amyloid Fibrils
6.8K
6.8K
01:29Subviral Agents
668
Subviral agents are infectious entities that resemble viruses but lack one or more viral components, such as a capsid or essential replication machinery. These agents include viroids, prions, and satellites, each possessing distinct structural and functional characteristics that influence their mode of infection and replication.Viroids are the simplest subviral agents, consisting of circular, single-stranded RNA molecules without a protein coat. They exclusively infect plants, relying entirely...
668