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Science 9 April 1982: Vol. 216. no. 4542, pp. 136 - 144 DOI: 10.1126/science.6801762
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Articles
Science, Vol 216, Issue 4542, 136-144
Copyright © 1982 by American Association for the Advancement of Science
Novel proteinaceous infectious particles cause scrapie
SB Prusiner
After infection and a prolonged incubation period, the scrapie agent causes a degenerative disease of the central nervous system in sheep and goats. Six lines of evidence including sensitivity to proteases demonstrate that this agent contains a protein that is required for infectivity. Although the scrapie agent is irreversibly inactivated by alkali, five procedures with more specificity for modifying nucleic acids failed to cause inactivation. The agent shows heterogeneity with respect to size, apparently a result of its hydrophobicity; the smallest form may have a molecular weight of 50,000 or less. Because the novel properties of the scrapie agent distinguish it from viruses, plasmids, and viroids, a new term "prion" is proposed to denote a small proteinaceous infectious particle which is resistant to inactivation by most procedures that modify nucleic acids. Knowledge of the scrapie agent structure may have significance for understanding the causes of several degenerative diseases.
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- Disease-related Prion Protein Forms Aggresomes in Neuronal Cells Leading to Caspase Activation and Apoptosis.
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- A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes.
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Science
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- The [URE3] Prion Is Not Conserved Among Saccharomyces Species.
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Genetics
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