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Novel proteinaceous infectious particles cause scrapie
Science (New York, N.Y.)
|April 9, 1982
Summary
The scrapie agent, a novel infectious particle, causes central nervous system degeneration. This protein-based agent, termed a prion, is resistant to nucleic acid inactivation, offering insights into degenerative diseases.
Area of Science:
- Neuroscience
- Infectious Diseases
- Biochemistry
Background:
- Scrapie is a degenerative central nervous system disease in sheep and goats.
- The infectious agent's nature has been a long-standing question in disease research.
Purpose of the Study:
- To characterize the scrapie agent responsible for the disease.
- To determine the molecular composition and properties of the infectious agent.
Main Methods:
- Sensitivity to proteases was assessed to evaluate protein content.
- Resistance to nucleic acid modification procedures was tested.
- Agent size and hydrophobicity were analyzed.
Main Results:
- The scrapie agent contains a protein essential for infectivity, indicated by protease sensitivity.
- The agent is resistant to inactivation by procedures targeting nucleic acids.
- The agent exhibits size heterogeneity, with the smallest form potentially having a molecular weight of 50,000 or less.
Conclusions:
- The scrapie agent is a novel infectious particle composed primarily of protein, distinct from viruses, plasmids, or viroids.
- A new term, "prion," is proposed for this small, proteinaceous infectious particle resistant to nucleic acid inactivation.
- Understanding prion structure may be crucial for comprehending various degenerative neurological diseases.