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In this groundbreaking paper, Stanley B. Prusiner presents evidence that the causative agent of scrapie, a neurodegenerative disease in sheep and goats, is an infectious protein particle, which he calls a “prion.” The resistance of the prion to procedures that modify nucleic acids, its heterogeneity in size and charge, and its partial purification are described. The hypothetical mechanisms of prion replication are discussed, considering the possibility that it lacks nucleic acid, and its possible involvement in other human neurodegenerative diseases is explored. The research proposes a new model for infectious agents, challenging the central dogma of molecular biology. Finally, the implications of understanding the structure and replication of prions for medicine are highlighted.
Prusiner, S. B. (1982). Novel proteinaceous infectious particles cause scrapie. Science, 216(4542), 136-144.
