- Person
- Stanley Prusiner
- Lived
- b. 1942
- Field
- Neurology
- From
- American
The story
Stanley Prusiner was born in 1942 into a Jewish family in Des Moines, Iowa, the son of an architect, and grew up there and in Cincinnati, Ohio. He studied chemistry and then medicine at the University of Pennsylvania, and learned to purify proteins during three years at the National Institutes of Health. In 1972, as a young neurology resident at the University of California, San Francisco, he lost a patient to Creutzfeldt-Jakob disease, a fatal brain disease that causes dementia, and set out to find what caused it and related illnesses such as scrapie in sheep.
At the time the agent was thought to be a mysterious slow virus, although some scientists had suggested it might lack genetic material. The work was slow and costly: each purification experiment needed scores of mice and a wait of about 200 days for symptoms to appear, until the team found that scrapie developed much faster in hamsters.
Ten years later, in 1982, he and his colleagues purified the infectious agent from diseased hamster brains. All the evidence showed it was a single protein, with none of the DNA or RNA that every known germ carried. He named it a prion, from proteinaceous infectious particle. The scientific community greeted the idea with great skepticism, but Prusiner kept going. He and Detlev Riesner searched harder than anyone for a hidden nucleic acid and never found one. His team later showed that the prion protein can fold into a normal shape or a harmful one, and that the harmful form converts normal copies in a chain reaction.
In 1997 he received the Nobel Prize in Physiology or Medicine for discovering prions, 'a new biological principle of infection'. The Nobel Assembly said he had added prions to the list of well-known infectious agents, alongside bacteria, viruses, fungi and parasites, and noted that his work could help explain other dementia-related diseases such as Alzheimer's and lay a foundation for new treatments.
His discovery explained the cause of scrapie, mad cow disease and human illnesses such as Creutzfeldt-Jakob disease, and he went on to found UCSF's Institute for Neurodegenerative Diseases in 1999 to apply the lessons of prions to more common brain diseases.
Why it matters
Prion science explained mad cow disease and related brain disorders, and it offers a basis for research into Alzheimer's and other dementia-related diseases.
About Stanley Prusiner
Neurologist who discovered prions, infectious proteins with no DNA or RNA, behind mad cow and Creutzfeldt–Jakob disease, winning the 1997 Nobel Prize for a once-derided idea.
More about Stanley Prusiner
- Stanley Prusiner showed prion disease can be inheritedStanley Prusiner's group showed that a mutation in the prion gene causes inherited brain disease, and reproduced the illness in genetically engineered mice.
- Stanley Prusiner founded an institute for brain diseasesIn 1999 Stanley Prusiner founded the Institute for Neurodegenerative Diseases at the University of California, San Francisco, to study illnesses caused by misfolded proteins.
- Stanley Prusiner told the prion story in a memoirIn 2014 Stanley Prusiner published Madness and Memory, his account of discovering prions and of the long scientific battle over an idea once dismissed as heresy.
- Stanley Prusiner found the prion gene in our own DNAIn 1984 Stanley Prusiner's team showed that the gene for the prion protein is found in all animals tested, including humans, a startling result that reshaped the understanding of brain disease.
- Stanley Prusiner's prions opened new routes to dementiaThe Nobel Assembly said Stanley Prusiner's discovery of prions could furnish the basis for understanding other dementias such as Alzheimer's disease and for developing new treatments.
- Stanley Prusiner revealed why prions are so hard to destroyStanley Prusiner's work showed that the disease-causing prion protein is extraordinarily stable, resisting enzymes, solvents and temperatures above 100°C, explaining why prions are so hard to kill.
Sources
- The Nobel Prize in Physiology or Medicine 1997: Press release NobelPrize.org
- Stanley B. Prusiner Wikipedia
- Stanley B. Prusiner: Biographical Nobel Prize
Another act of tikkun olam. This is part of tikkun olam, the Jewish idea of repairing the world, and every one of us can add to it.