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Stanley Prusiner

b. 1942 Specialist

The neurologist who said an infection could be pure protein

Portrait of Stanley Prusiner
Christopher Michel — 2024 — CC BY 4.0 via Wikimedia Commons

Stanley Prusiner grew up in Des Moines and then Cincinnati, the son of an architect, and picked up the nickname 'the little genius' at Walnut Hills High School for a science-fair project on repelling boxelder bugs. He studied chemistry at the University of Pennsylvania, stayed on for a medical degree, then spent time at the National Institutes of Health under biochemist Earl Stadtman studying enzymes before an internship pulled him to the University of California, San Francisco. He finished a neurology residency there in 1974 and joined the faculty the same year, with no plan yet to spend the next two decades arguing that an infectious disease could travel without a single strand of DNA or RNA attached to it.

By 1982 Prusiner was arguing, against the field's basic assumptions, that scrapie and Creutzfeldt-Jakob disease spread through a purely protein pathogen with no genetic material — breaking what biology treated as settled law, since nothing was supposed to replicate without DNA or RNA to carry instructions. He coined a name for it, prion, and spent much of the 1980s treated as a crank for it, especially once mad cow disease in Britain put the stakes in front of the public. Genetic and structural evidence eventually came down on his side: a misfolded protein can force normal copies of itself to misfold too, and the process spreads through tissue like an infection. He won the 1997 Nobel Prize in Physiology or Medicine alone and has directed UCSF's Institute for Neurodegenerative Diseases since 1999, since extending the prion mechanism toward Alzheimer's research.

Prusiner spent most of the 1980s treated, by his own account and colleagues', as something close to a crank. The journal Cell rejected his central paper; it ran instead in the Proceedings of the National Academy of Sciences, which skips outside peer review, and grant reviewers who wanted funding to keep hunting for a conventional virus were told the idea lacked scientific merit. The skepticism did not end with the 1997 Nobel Prize — Science and Nature published papers challenging the prion hypothesis right up to the announcement, and critics wrote that the committee had rewarded conviction over proof, warning that if a slow virus turned out to be the real cause, Prusiner would have won for discovering something spectacularly wrong. Three decades of structural and genetic evidence have since gone against the critics.

The concept he was ridiculed for has since become its own field. 'Prion-like' mechanisms, misfolded proteins recruiting normal copies of themselves into disease, are now studied across Alzheimer's, Parkinson's, and ALS research, and Prusiner has spent the years since his Nobel directing the UCSF institute built around that idea. He collected the National Medal of Science in 2010 and the Lasker and Wolf Prizes before that. At 83, he remains an active researcher rather than an emeritus figurehead, and the scientific argument that once tried to sink him — that no infectious agent could work without DNA or RNA — is the one that ended up revised.

Lifespan

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Stanley Prusiner's life against the full span of the corpus — the fading end marks a life still in progress.

Notable works

  • Novel proteinaceous infectious particles cause scrapie article
    Science — coined the term 'prion'
    1982
  • Identification of PrP, the prion protein discovery 1984
  • UCSF Institute for Neurodegenerative Diseases company
    Prusiner has directed it since founding
    1999
  • Nobel Lecture: Prions speech
    Delivered in Stockholm
    1997
  • Madness and Memory: The Discovery of Prions book
    Yale University Press
    2014

Life in brief

with age at each point
  • May 28, 1942 aged 0 Born in Des Moines, Iowa
  • 1974 aged ~32 Finishes his neurology residency at UCSF and joins the faculty
  • 1982 aged ~40 Publishes the prion hypothesis in Science Coined the term 'prion,' met with widespread scientific skepticism.
  • 1996 aged ~54 The U.K. links mad cow disease to human variant CJD Brought public attention to the prion hypothesis Prusiner had been defending for over a decade.
  • October 6, 1997 aged 55 Awarded the Nobel Prize in Physiology or Medicine, alone
  • 1999 aged ~57 Founds and begins directing UCSF's Institute for Neurodegenerative Diseases
  • 2010 aged ~68 Awarded the National Medal of Science
  • 2014 aged ~72 Publishes the memoir Madness and Memory

Related

Contemporaries

Science & Medicine, born within 25 years
Nobel Prize