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Updated: May 6, 2026

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
Published on: January 8, 2015
Infectivity versus Seeding in Neurodegenerative Diseases Sharing a Prion-Like Mechanism
Natalia Fernández-Borges1, Hasier Eraña, Saioa R Elezgarai
1CIC bioGUNE, Parque Tecnológico de Bizkaia, Derio, 48160 Bizkaia, Spain.
Abstract:
Prions are considered the best example to prove that the biological information can be transferred protein to protein through a conformational change. The term "prion-like" is used to describe molecular mechanisms that share similarities with the mammalian prion protein self-perpetuating aggregation and spreading characteristics. Since prions are presumably composed only of protein and are infectious, the more similar the mechanisms that occur in the different neurodegenerative diseases, the more these processes will resemble an infection. In vitro and in vivo experiments carried out during the last decade in different neurodegenerative disorders such as Alzheimer's disease (AD), Parkinson's diseases (PD), and amyotrophic lateral sclerosis (ALS) have shown a convergence toward a unique mechanism of misfolded protein propagation. In spite of the term "infection" that could be used to explain the mechanism governing the diversity of the pathological processes, other concepts as "seeding" or "de novo induction" are being used to describe the in vivo propagation and transmissibility of misfolded proteins. The current studies are demanding an extended definition of "disease-causing agents" to include those already accepted as well as other misfolded proteins. In this new scenario, "seeding" would be a type of mechanism by which an infectious agent can be transmitted but should not be used to define a whole "infection" process.
Insights
Prion-like mechanisms involve protein misfolding and propagation in neurodegenerative diseases like Alzheimer's and Parkinson's. These processes, similar to prion infection, highlight the need for broader definitions of disease-causing agents.
Area of Science:
- Neuroscience
- Molecular Biology
- Pathology
Background:
- Prions exemplify protein-to-protein biological information transfer via conformational changes.
- The term "prion-like" describes mechanisms mimicking prion self-perpetuating aggregation and spreading.
- Neurodegenerative diseases share convergent mechanisms of misfolded protein propagation.
Purpose of the Study:
- To explore the concept of "prion-like" behavior in neurodegenerative disorders.
- To discuss the implications of protein misfolding propagation for disease mechanisms.
- To propose an extended definition of disease-causing agents.
Main Methods:
- Review of in vitro and in vivo experiments on Alzheimer's disease, Parkinson's disease, and ALS.
- Analysis of molecular mechanisms of protein aggregation and spreading.
- Conceptual analysis of prion infection, seeding, and de novo induction.
Main Results:
- Evidence shows a convergence toward a common mechanism of misfolded protein propagation across different neurodegenerative diseases.
- The term "infection" can describe these processes, alongside "seeding" and "de novo induction" for in vivo propagation.
- Current research necessitates an expanded definition of "disease-causing agents" to encompass various misfolded proteins.
Conclusions:
- Misfolded protein propagation is a unifying mechanism in neurodegenerative diseases.
- "Seeding" is a transmission mechanism but not the entirety of an "infection" process.
- The definition of disease-causing agents should evolve to include diverse misfolded proteins involved in prion-like phenomena.
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