- Person
- Stanley Prusiner
- Lived
- b. 1942
- Field
- Neurology
- From
- American
The story
Stanley Prusiner had long argued that prions might shed light on far more common disorders than Creutzfeldt-Jakob disease. His discovery that prions can assemble into the long fibres known as amyloid led him to propose that Alzheimer's and Parkinson's diseases might also be caused by prions, misfolded proteins that spread their faulty shape from molecule to molecule.
To pursue that idea on a larger scale, in 1999, two years after his Nobel Prize, he founded the Institute for Neurodegenerative Diseases at the University of California, San Francisco, and became its founding director. The institute is now based in the Sandler Neurosciences Center at UCSF.
The institute conducts research on neurodegenerative diseases caused by the accumulation of misfolded proteins. Its research areas include Alzheimer's disease, the synucleinopathies such as Parkinson's disease, the tauopathies and prion diseases such as Creutzfeldt-Jakob disease. According to the institute, evidence has continued to accumulate that prions are involved not only in Alzheimer's and Parkinson's but also in amyotrophic lateral sclerosis, the frontotemporal dementias, chronic traumatic encephalopathy and multiple system atrophy.
The institute's stated goal is practical. It points out that there is still no medicine that halts or even slows any neurodegenerative disease, and it aims to develop drugs that stop prions from accumulating and so halt the destruction of nerve cells. Much of Prusiner's own current research focuses on developing therapies that reduce the levels of the specific prions responsible for these illnesses, and on new ways of diagnosing them. The institute also trains young scientists through a postdoctoral fellowship programme.
The institute reflects the path of Prusiner's whole career. A question first raised by a single patient with a rare disease in 1972 grew into a research programme aimed at some of the most widespread and devastating illnesses of old age. In the institute's words, its aim is to end the day when a neurologist gives a patient a diagnosis of one of these diseases but can offer no hope.
Why it matters
The institute brings the lessons of prion research to bear on Alzheimer's, Parkinson's and other neurodegenerative diseases that affect millions of families.
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 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.
- 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
- Stanley B. Prusiner Wikipedia
- Stanley B. Prusiner, MD UCSF Institute for Neurodegenerative Diseases
- Institute for Neurodegenerative Diseases University of California, San Francisco
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.