The prion hypothesis is finally accepted by the establishmentBMJ 1997; 315 doi: https://doi.org/10.1136/bmj.315.7114.967l (Published 18 October 1997) Cite this as: BMJ 1997;315:967
- Deborah Josefson
Professor Stanley Prusiner has won this year's Nobel prize for medicine for his pioneering work on prions. Deborah Josefson examines his groundbreaking and frequently controversial research
Stanley Prusiner, professor of neurology and biochemistry at the University of California, San Francisco, has won the 1997 Nobel prize for medicine for his work on prions. This new class of infectious agents are thought to underlie a variety of sporadic, transmissible, and hereditary neurodegenerative disorders including kuru, Gerstmann-Sträussler-Scheinker disease, fatal familial insomnia, Creutzfeldt-Jakob disease (CJD), scrapie, and bovine spongiform encephalopathy (BSE).
Professor Prusiner's work has long been controversial because it challenges conventional biomedical dogma which holds that an infectious agent must contain nucleic acid. The Nobel prize winner's revolutionary concept was that a deformed protein could serve as an infectious particle and propagate in the absence of genetic material. Professor Prusiner dubbed these proteins “prions”-short for proteinaceous infectious particles.
The Karolinska Institute, which bestowed the prize, said: “Stanley Prusiner has added prions to the list of well known infectious agents including bacteria, viruses, fungi, and parasites. Stanley Prusiner's discovery provides important insights that may furnish the basis of understanding the biological mechanisms underlying other types of dementia related illnesses-for example, Alzheimer's …