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Published on: November 6, 2013
Prion diseases: Lessons from historical outbreaks and potential emerging ones
Aidan P Holman1,2, Dmitry Kurouski1,3
1Department of Biochemistry and Biophysics, Texas A&M University, College Station, Texas, USA.
Abstract:
Prion diseases (PrDs) are a unique and fatal class of neurodegenerative disorders caused by misfolded proteinaceous infectious particles, or prions. While the pathogenic form was first documented in humans nearly a century ago, the global monitoring of PrDs only gained momentum after the "Mad Cow" epizootic and its human counterpart of the 1980s and 1990s. Currently, 34 countries track human prion cases annually, with over 27,000 cases. However, true prevalence estimates suggest significantly higher numbers, millions, highlighting the urgency of addressing these enigmatic diseases. Prions are exceptionally resilient, resisting conventional sterilization methods and persisting in environmental reservoirs, such as soil and plants, raising concerns about environmental and cross-species transmission, particularly with the growing prevalence of chronic wasting disease (CWD) in cervids. This review explores the history, pathogenesis, presence, public health implications, and novel innovations in studying and treatment of PrDs. Future priorities should include the development of faster, cost-effective diagnostic tools and systemic therapies to neutralize prions in affected individuals and mitigate environmental risks. Understanding and addressing the challenges posed by prions is critical for global health security in the wake of CWD.
Insights
Prion diseases (PrDs) are fatal neurodegenerative disorders caused by prions. This review covers PrD history, pathogenesis, and public health risks, emphasizing urgent needs for diagnostics and therapies to combat these resilient infectious agents.
Area of Science:
- Neurodegenerative Disorders
- Infectious Diseases
- Molecular Biology
Background:
- Prion diseases (PrDs) are fatal neurodegenerative conditions caused by misfolded prions.
- Global monitoring of PrDs intensified after the "Mad Cow" disease outbreak.
- Over 27,000 human cases are tracked annually, but millions are estimated globally.
Purpose of the Study:
- To review the history, pathogenesis, and public health implications of prion diseases.
- To explore novel innovations in the study and treatment of PrDs.
- To highlight the urgent need for improved diagnostics and therapies.
Main Methods:
- Literature review of prion disease history and research.
- Analysis of global case tracking data and prevalence estimates.
- Exploration of prion resilience and environmental persistence.
Main Results:
- Prions are exceptionally resilient, resisting sterilization and persisting in the environment.
- Chronic Wasting Disease (CWD) in cervids raises concerns for cross-species transmission.
- Significant gaps exist in current diagnostic capabilities and treatment options.
Conclusions:
- Addressing prion diseases is critical for global health security.
- Development of rapid, cost-effective diagnostics is a priority.
- Systemic therapies are needed to neutralize prions and mitigate environmental risks.
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