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Publication : Novel proteinaceous infectious particles cause scrapie.

First Author  Prusiner SB Year  1982
Journal  Science Volume  216
Issue  4542 Pages  136-44
PubMed ID  6801762 Mgi Jnum  J:42977
Mgi Id  MGI:1096819 Doi  10.1126/science.6801762
Citation  Prusiner SB (1982) Novel proteinaceous infectious particles cause scrapie. Science 216(4542):136-44
abstractText  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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