- Person
- Stanley Prusiner
- Lived
- b. 1942
- Field
- Neurology
- From
- American
The story
When the Nobel Assembly honoured Stanley Prusiner in 1997, it looked beyond the rare prion diseases such as Creutzfeldt-Jakob disease, which affects about one person in a million. His work, it said, provides important insights that may furnish the basis to understand the mechanisms of other dementia-related diseases, for example Alzheimer's disease, and establishes a foundation for drug development and new types of medical treatment strategies.
The key insight was that a protein can cause disease simply by folding into the wrong shape and then forcing other copies of the protein to misfold too. Prions are not poisonous in themselves. They become harmful by changing their structure, sticking together into threads and clumps that eventually destroy nerve cells. Different variants build up in different parts of the brain, which explains why prion diseases can affect memory, movement or sleep. The Assembly noted indications that Alzheimer's disease arises when certain other proteins change shape and form harmful deposits in the brain.
Prusiner's work also suggested a strategy for treatment: drugs that prevent the conversion of normal prion proteins into the disease-causing form.
Prusiner himself pursued the connection. Building on his finding that prions can assemble into amyloid fibres, the same kind of deposit seen in Alzheimer's, he proposed that the more common neurodegenerative diseases may be caused by prions. In 1999 he founded the Institute for Neurodegenerative Diseases at the University of California, San Francisco, to study illnesses caused by misfolded proteins. According to the institute, a growing body of evidence links prion-like proteins not only to Alzheimer's and Parkinson's diseases but also to amyotrophic lateral sclerosis, the frontotemporal dementias, chronic traumatic encephalopathy and multiple system atrophy, and much of his current research aims to develop drugs that lower the levels of these specific prions.
Research by many groups has since explored how misfolded proteins spread in Alzheimer's, Parkinson's and related conditions, a field in which Prusiner's ideas remain influential. For illnesses that still have no treatment able to halt them, this line of research offers a clear target for future medicines.
Why it matters
The idea that misfolded proteins drive disease has become central to research on Alzheimer's and Parkinson's, conditions that affect tens of millions of people.
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 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, MD UCSF Institute for Neurodegenerative Diseases
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.