- Person
- Stanley Prusiner
- Lived
- b. 1942
- Field
- Neurology
- From
- American
The story
Long before prions were understood, scientists knew that the agent behind scrapie and Creutzfeldt-Jakob disease was unusually tough. It survived treatments that destroyed ordinary viruses and bacteria, and it did not provoke the body's normal defences against infection.
Stanley Prusiner's research explained why. The infectious agent is not a microbe but a misfolded form of the prion protein, which the body makes naturally. As the Nobel Assembly described in 1997, this disease-causing form is extremely stable and resistant to protein-digesting enzymes, organic solvents and high temperatures, even above 100°C. Once formed, it can trigger a chain reaction in which normal copies of the protein are converted into the stable harmful form. Because the protein is a natural part of the body, the immune system does not react to it.
This understanding mattered urgently in the late 1980s and 1990s, when an epidemic of bovine spongiform encephalopathy, or mad cow disease, swept British cattle. The epidemic first became evident in 1985 and spread through cattle feed containing offal from infected sheep. Because of the long incubation time, it did not peak until 1992, when about 37,000 animals were affected in that year alone. From 1995 a new variant of Creutzfeldt-Jakob disease appeared in people, which may have arisen through transmission of BSE.
Medicine had also seen the danger. In rare cases, Creutzfeldt-Jakob disease had been passed on through growth hormone extracted from human pituitary glands and through transplants of brain membrane tissue. Knowing that prions resist ordinary sterilisation helped scientists and health authorities understand the risks posed by contaminated food and medical procedures.
Prusiner's explanation of why prions are so hard to destroy gave a scientific basis for these precautions. It showed that the threat came not from a hidden virus but from a remarkably durable protein, and that preventing prion disease meant keeping infected material out of food and medicine in the first place.
Why it matters
Knowledge of how resistant prions are has informed safety practices for food, blood products and surgical instruments, protecting patients from accidental transmission.
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 discovered prions, a new kind of infectionIn 1982 Stanley Prusiner identified prions, infectious proteins with no DNA or RNA, an idea met with scepticism that won him the 1997 Nobel Prize.
- 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.
Sources
- The Nobel Prize in Physiology or Medicine 1997, Press release NobelPrize.org
- Stanley B. Prusiner Wikipedia
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.