Stanley B. Prusiner

Stanley B. Prusiner is an American neurologist and biochemist who discovered prions, infectious agents composed primarily of misfolded proteins. He received the 1997 Nobel Prize in Physiology or Medicine.

Prion: An infectious agent composed primarily of a misfolded protein that can induce similar misfolding in normal proteins. Prusiner named and characterized the protein-based infectious agent at the center of his work.
Kuru: A fatal neurodegenerative disease historically transmitted among the Fore people of Papua New Guinea. Research into kuru helped establish that human prion diseases could be transmissible.
Carleton Gajdusek: An American physician and scientist who studied kuru and other transmissible spongiform encephalopathies. Gajdusek’s kuru research established transmission as a key problem Prusiner sought to explain.
Creutzfeldt–Jakob disease: A rare, rapidly progressive human neurodegenerative disease caused by prions. Prusiner studied this human prion disease and helped explain its infectious biology.
Virus: An infectious agent that replicates inside host cells using genetic material enclosed in a protein coat or membrane. Prusiner argued that prions transmit infection without the nucleic-acid genome characteristic of viruses.
Prion protein: A host-encoded protein that occurs in normal and disease-associated forms and is central to prion disorders. Prusiner’s hypothesis linked disease transmission to changes in this protein’s shape.
Scrapie: A fatal neurodegenerative disease of sheep and goats, recognized for centuries and transmissible between animals. Experiments on scrapie provided a model for investigating the unusual infectious agent Prusiner proposed.
Tikvah Alper: A radiation biophysicist who investigated the nature of the scrapie infectious agent. Alper’s work supported the idea that scrapie infectivity lacked a conventional nucleic-acid genome.
Variant Creutzfeldt–Jakob disease: A human prion disease linked to exposure to the agent causing bovine spongiform encephalopathy. Its emergence demonstrated the public-health consequences of prion transmission across species.
Viroid: A small infectious RNA molecule that causes disease in plants without a protein coat. Viroids show that infectious agents can lack protein coats, but still depend on nucleic acid.

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