Stanley B. Prusiner, M.D. is Director of the Institute for Neurodegenerative Diseases and Professor of Neurology at the University of California San Francisco. Editor of 11 books and author of over 500 research articles, Dr. Prusiner’s contributions to scientific research are internationally recognized.
Prusiner discovered an unprecedented class of pathogens that he named prions. Prions are proteins that acquire an alternative shape that becomes self-propagating. As prions accumulate, they cause neurodegenerative diseases in animals and humans. Prusiner’s discovery led him to develop a novel disease paradigm: prions cause disorders such as Creutzfeldt-Jakob disease (CJD) in humans that manifest as (1) sporadic, (2) inherited and (3) infectious illnesses. Many scientists considered Prusiner’s concept of “infectious proteins” as well as his proposal that a single protein could possess multiple biologically active shapes or conformations to be heretical. Based on his seminal discovery that prions can assemble into amyloid fibrils, Prusiner proposed that neurodegenerative diseases including Alzheimer’s and Parkinson’s may be caused by prions. Remarkably, a wealth of evidence continues to accumulate arguing that prions cause not only these common degenerative diseases, but also the frontotemporal dementias (FTDs), chronic traumatic encephalopathy (CTE), multiple system atrophy (MSA) and dementia with Lewy bodies (DLB). Much of Prusiner’s current research focuses on developing therapeutics that reduce the levels of the specific prions responsible for MSA, DLB, and some FTDs as well as CTE.
Among his many honors for his groundbreaking work, Dr. Prusiner was awarded the Nobel Prize in Physiology or Medicine (1997) and the United States National Medal of Science (2009).