Tuesday, February 23, 2010

Stanley Prusiner

If you win a Nobel prize you get not only a nice medal, a posh banquet in Sweden, over a million bucks and the adulation of your peers but you also get to write your own biography for publication. These are actually quite interesting and are all available at the Nobel Foundation website.

Here are some excerpts from Stanley Prusiner's biography.

In July 1972, I began a residency at the University of California San Francisco in the Department of Neurology. Two months later, I admitted a female patient who was exhibiting progressive loss of memory and difficulty performing some routine tasks. I was surprised to learn that she was dying of a "slow virus" infection called Creutzfeldt-Jakob disease (CJD) which evoked no response from the body's defenses. Next, I learned that scientists were unsure if a virus was really the cause of CJD since the causative infectious agent had some unusual properties. The amazing properties of the presumed causative "slow virus" captivated my imagination and I began to think that defining the molecular structure of this elusive agent might be a wonderful research project. The more that I read about CJD and the seemingly related diseases - kuru of the Fore people of New Guinea and scrapie of sheep - the more captivated I became.
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I had anticipated that the purified scrapie agent would turn out to be a small virus and was puzzled when the data kept telling me that our preparations contained protein but not nucleic acid. About this time, I was informed by the Howard Hughes Medical Institute (HHMI) that they would not renew their support and by UCSF that I would not be promoted to tenure. When everything seemed to be going wrong, including the conclusions of my research studies, it was the unwavering, enthusiastic support of a few of my closest colleagues that carried me through this very trying and difficult period.
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As the data for a protein and the absence of a nucleic acid in the scrapie agent accumulated, I grew more confident that my findings were not artifacts and decided to summarize that work in an article that was eventually published in the spring of 1982. Publication of this manuscript, in which I introduced the term "prion", set off a firestorm. Virologists were generally incredulous and some investigators working on scrapie and CJD were irate.

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