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Scrapie and Creutzfeldt-Jakob disease prions
S B Prusiner1, R A Barry, M P McKinley
1Department of Neurology, University of California, San Francisco 96143.
Microbiological Sciences
|January 1, 1985
Summary
Prions cause diseases like Creutzfeldt-Jakob disease and scrapie. These proteinaceous infectious agents form amyloid plaques in the brain, similar to viral inclusion bodies, indicating they are the direct cause of disease.
Area of Science:
- Neuroscience
- Pathology
- Infectious Diseases
Background:
- Scrapie and Creutzfeldt-Jakob disease are transmissible spongiform encephalopathies.
- The causative agents, prions, are distinct from viruses and viroids.
Purpose of the Study:
- To characterize the nature of prions and their aggregation in the brain.
- To differentiate prion diseases from viral and viroid infections.
Main Methods:
- Ultrastructural analysis of prion aggregates.
- Histochemical examination of brain tissue.
Main Results:
- Prion aggregates are ultrastructurally and histochemically identical to amyloid.
- Extracellular prion collections form amyloid plaques in scrapie-infected brains.
Conclusions:
- Prion amyloid plaques are composed of the causative pathogen.
- These plaques are analogous to viral inclusion bodies, representing the disease's cause, not just a consequence.