Related Experiment Video
Updated: Aug 31, 2025

10:40
Author Spotlight: Insight Into Advances in Prion Diseases Research
Published on: August 11, 2023
557
Recent advances in cellular models for discovering prion disease therapeutics
Lea Nikolić1, Chiara Ferracin1, Giuseppe Legname1
1Laboratory of Prion Biology, Department of Neuroscience, Scuola Internazionale Superiore di Studi Avanzati (SISSA), Trieste, Italy.
Expert Opinion on Drug Discovery
|August 19, 2022
Summary
Prion diseases are fatal neurodegenerative conditions caused by misfolded prion proteins. Research increasingly uses cell-based models for faster, ethical drug discovery, though careful model selection is crucial.
Area of Science:
- Neurodegenerative Diseases
- Prion Biology
- Cellular Models
Background:
- Prion diseases result from the misfolding of cellular prion protein into infectious prions.
- These agents are highly resistant to inactivation and cause progressive brain damage.
- Current treatments for human prion diseases are limited, necessitating novel therapeutic strategies.
Purpose of the Study:
- To review the current landscape of prion research models.
- To highlight the utility of cell-based models as alternatives to animal models.
- To guide the selection of appropriate cellular models for drug screening.
Main Methods:
- Review of existing literature on prion disease research models.
- Discussion of various prion-infected cell lines and their applications.
- Analysis of the benefits and limitations of different cellular models.
Main Results:
- Cell-based models offer a faster and more ethical approach to studying prion diseases.
- Animal models remain essential but are complemented by in vitro systems.
- No single cellular model is universally ideal; selection depends on research goals.
Conclusions:
- Cellular models are invaluable tools for understanding prion pathogenesis and developing therapeutics.
- Careful consideration of model-specific advantages and disadvantages is essential for successful drug discovery.
- Further development of relevant cellular models is critical for advancing prion disease research.
Related Concept Videos
Amyloid Fibrils
9.8K
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,...
9.8K
EPS and iPS Cells in Disease Research
2.8K
Embryonic and induced pluripotent stem cells are excellent models for disease research because of their ability to self-renew and differentiate into most cell types. Somatic cells from a patient are isolated and reprogrammed into induced pluripotent stem cells or iPSCs. These iPSCs are later differentiated into the desired cell type, which mirrors the diseased cell of the patient. In this way, disease models have been created for investigating diseases such as Down syndrome, type I diabetes,...
2.8K
Subviral Agents
87
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...
87

