PRUSINER, Stanley Benjamin
Novel proteinaceous infectious particles cause Scrapie.Science, 216 (4542),136–144., 1982.
Prusiner won the Nobel Prize in Physiology or Medicine in 1997 for his work in proposing a completely novel explanation for the cause of bovine spongiform encephalopathy ("mad cow disease") and its human equivalent, Creutzfeldt–Jakob disease.
To describe the cause of the disease in this paper published on April 9, 1982 Prusiner coined the term prion, which comes from the words "proteinaceous" and "infectious," to refer to a previously undescribed form of infection, due to protein misfolding, with no DNA or RNA involved. This new concept "violated all the rules" and failed to convince the scientific community, most of whom initially thought that Prusiner was "totally insane."
Subjects: INFECTIOUS DISEASE › Prion Diseases, NEUROLOGY › Degenerative Disorders, VETERINARY MEDICINE
Identification of a protein that purifies with the Scrapie prion.Science, 218, 1309-1311, 1982.
Research with the biochemist Bolton enabled Prusiner to discover and characterize the specific protein causing prion disease. This paper was dated December 24, 1982.
Nearly simultaneously, Prusiner and the same co-authors published an additional, longer paper dated December 21, 1982, further characterizing the pure protein of the infectious agent:
"Further purification and characterization of Scrapie prions," Biochemistry, 21 (1982) 6942-6950.
These papers provided the necessary evidence to convince members of the scientific community, and eventually led to Prusiner's Nobel Prize.
(Thanks to Juan Weiss for these references and their interpretation.)
Subjects: INFECTIOUS DISEASE › Prion Diseases, VETERINARY MEDICINE
Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity.Science, 274, 2079-2082, 1996.
The authors showed that the " 'normal prion protein' in the brains of living mice can be converted into different forms depending on the type of abnormal human prion that initiated the conversion. The result is different patterns of pathological changes in the host, as would be expected for different prion strains." Prusiner proposed that "infection with one of the misfolded proteins can induce disease by forcing healthy PrP molecules to refold themselves into abnormal prions."
Subjects: BIOLOGY › MOLECULAR BIOLOGY › Protein Synthesis, INFECTIOUS DISEASE › Prion Diseases
Synthetic mammalian prions.Science, 305, 673-676, 2004.
The authors modified Koch's Postulates within the context of prion disease. To do so the followed these steps:
Subjects: INFECTIOUS DISEASE › GENERAL PRINCIPLES of Infection by Microorganisms, INFECTIOUS DISEASE › Prion Diseases
Madness and memory: The discovery of prions- a new biological principle of disease.New Haven, CT: Yale University Press, 2014.
Prusiner discovered prions, the agent causing scrapie in sheep and goats, mad cow disease, and Creutzfeldt-Jakob disease in humans.
Subjects: BIOGRAPHY (Reference Works) › Autobiography, INFECTIOUS DISEASE › History of Infectious Disease, INFECTIOUS DISEASE › Prion Diseases, NEUROLOGY › Degenerative Disorders, NEUROSCIENCE › Neuropsychology › Memory, VETERINARY MEDICINE