Prusiner's Prions

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While everyone loves the romantic notion of the scientific revolutionary who bucks a doubting “establishment” to change our understanding of the world, every now and then, that narrative comes true. In the early 1970s, an otherwise healthy woman became a patient in the University of California, San Francisco’s neurology department; she was suffering from something called a “slow virus infection,” specifically, a neurodegenerative condition called Creutzfeldt-Jakob disease.

Her case caught the attention of a scientist named Stanley Prusiner, who watched her go from a normal state of being able to walk, talk and carry out coordinated movements, to a state of advanced dementia in which she was mute, unable to walk, or care for herself to any degree. Within a few months, she was dead.

Prusiner became fascinated by this medical mystery, particularly the fact that such infections — which include rare disorders such as kuru, mad cow disease, scrapie (in sheep), and Alzheimer’s disease — don’t respond to any known treatment. He set out to find out what was causing these diseases. And ten years later, he had his answer: the culprit was a single protein, which Prusiner dubbed a “prion.”

The weird thing about this agent is that most viruses/infectious agents contain either DNA or RNA; prions seemed to be pure proteins. The scientific community reacted with extreme skepticism, even hostility, to Prusiner’s hypothesis. Yet Prusiner and several colleagues persevered, continued their research, and by the 1990s, the existence of prions was far more scientifically acceptable. Prusiner won the 1997 Nobel Prize in Physiology or Medicine for his pioneering research.

Prusiner was one of the featured speakers at a recent salon sponsored by The Science and Entertainment Exchange, where he told the assembled leaders in the entertainment industry all about prions and the deadly diseases they cause. But he also believes these diseases can be cured, although it will require some novel approaches to developing effective new drugs. That’s hopeful news for those with family members who have suffered and died from this class of disease.

The statements and opinions expressed in this piece are those of the event participants and do not necessarily reflect the views of any organization or agency that provided support for this event or of the National Academies of Sciences, Engineering, and Medicine.