Before winning his Nobel prize, Stanley Prusiner was ridiculed for suggesting that something he called a prion caused spongiform brain diseases
WHEN the evidence suggested that the baffling 鈥渟pongiform鈥� brain disorders Creutzfeldt-Jakob disease (CJD), kuru and scrapie could not be transmitted by a virus or bacterium, the neurologist Stanley Prusiner put forward a novel type of infectious agent as the cause: a rogue protein. It was an idea considered so outrageous that Prusiner was ridiculed.
Prusiner first began to study these diseases in 1972, after one of his patients at the University of California, San Francisco, died of CJD. A decade later, in the journal Science (vol 216, p 136), he suggested that these diseases were caused by a 鈥溾��, or prion.
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The idea built on the findings of British researchers. In 1967, of the Medical Research Council鈥檚 Radiopathology Unit showed that whatever it was that caused CJD was unscathed by levels of ultraviolet radiation that would destroy any genetic material (Nature, vol 214, p 764). Shortly afterwards, mathematician of Bedford College in London devised a protein-only hypothesis for scrapie propagation. His 1967 Nature paper (vol 215, p 1043) states there was no reason to fear that the idea 鈥渨ould cause the whole theoretical structure of molecular biology to come tumbling down鈥�.
This work sparked little interest when it was published. By the time Prusiner became involved, however, indifference had hardened into scepticism. In December 1986, a sardonic profile of Prusiner appeared in Discover magazine, headed 鈥淭he name of the game is fame: but is it science?鈥� Yet just 11 years later, Nobel prize. There are still unanswered questions about the prion model, but no one doubts that Prusiner鈥檚 work provides deeper understanding of this cause of dementia.
Read more: Zeros to heroes: 10 unlikely ideas that changed the world
