Related Experiment Video
Updated: Jan 19, 2026

10:40
Author Spotlight: Insight Into Advances in Prion Diseases Research
Published on: August 11, 2023
815
Introduction to Current Progress in Advanced Research on Prions
Takashi Onodera1, Akikazu Sakudo2
1Research Center for Food Safety, Graduate School of Agricultural and Life Sciences, University of Tokyo, Tokyo, Japan.
Current Issues in Molecular Biology
|September 28, 2019
Summary
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are fatal neurological conditions. This review explores how prion protein aggregates cause these diseases and bypass the body's defenses.
Area of Science:
- Neuroscience
- Infectious Diseases
- Molecular Biology
Background:
- Prion diseases, including Creutzfeldt-Jakob disease (CJD) and bovine spongiform encephalopathy (BSE), are fatal neurodegenerative disorders.
- A central event is the misfolding of the prion protein (PrPC) into an abnormal, infectious form (PrPSc).
- These diseases can be sporadic, genetic (due to PrP gene mutations), or infectious.
Purpose of the Study:
- To provide an overview of current research in prion disease studies.
- To highlight the mechanisms of prion disease transmission and pathogenesis.
- To address the challenges in understanding how prions cross biological barriers.
Main Methods:
- This is a review article, summarizing existing research and knowledge on prion diseases.
- It discusses the conversion of PrPC to PrPSc.
- It examines various routes of prion disease transmission and the challenges in studying them.
Main Results:
- Prion diseases manifest in various species, including humans, cattle, and cervids.
- Prion diseases can arise from genetic mutations, spontaneous misfolding, or external exposure to prions.
- The mechanisms by which prions spread and cause disease, particularly crossing the blood-brain barrier, remain areas of active investigation.
Conclusions:
- Understanding prion disease pathogenesis is crucial for developing effective diagnostics and therapeutics.
- Further research is needed to elucidate the complex interactions between prions and the host.
- Addressing the challenges of prion transmission and barrier crossing is key to combating these devastating diseases.
Related Concept Videos
Amyloid Fibrils
11.7K
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,...
11.7K
Subviral Agents
534
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...
534

