This story begins in the wild country of the highlands of Papua New Guinea in the 1950s and ends in a research laboratory in California where prions were first discovered. Dr. Carleton Gajdusek (GHY-da-shek), the brilliant but deeply flawed pediatrician, intrigued by his years studying rabies, plague, scurvy, and arbovirus infections, wanted to find a rare disease that he could call his own. He found this disease—kuru—in Papua New Guinea. After years of studying victims of this disease, he finally declared in the 1970s that an unconventional slow virus caused the disease that was spread by cannibalism. He received the Nobel Prize in 1976 for his work on new mechanisms for the dissemination of infectious diseases. Dr. Stanley Prusiner, a neurologist at the University of California at San Francisco, emerged in the 1970s, with the goal of identifying the infectious agent which by this time included several diseases in addition to kuru now called transmissible spongiform encephalopathies (TSEs): Enkephalos from the Greek for brain and pathos meaning disease. His work led to the theory that the infectious agent was not a virus as proposed by Gajdusek but was a protein which he called a prion. The story of these two Nobelists is clouded by the personalities of each awardee: Gajdusek was described as a scientific genius with the emotional maturity of a child. He would eventually end up disgraced and in exile from the U.S. Prusiner was described by his colleagues as aggressive and egotistical, and as one colleague put it, he had never seen anybody covet the Nobel as openly as Prusiner.

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The Prion Diseases

  • Virginia Shepherd,
  • Charles Brau

摘要

This story begins in the wild country of the highlands of Papua New Guinea in the 1950s and ends in a research laboratory in California where prions were first discovered. Dr. Carleton Gajdusek (GHY-da-shek), the brilliant but deeply flawed pediatrician, intrigued by his years studying rabies, plague, scurvy, and arbovirus infections, wanted to find a rare disease that he could call his own. He found this disease—kuru—in Papua New Guinea. After years of studying victims of this disease, he finally declared in the 1970s that an unconventional slow virus caused the disease that was spread by cannibalism. He received the Nobel Prize in 1976 for his work on new mechanisms for the dissemination of infectious diseases. Dr. Stanley Prusiner, a neurologist at the University of California at San Francisco, emerged in the 1970s, with the goal of identifying the infectious agent which by this time included several diseases in addition to kuru now called transmissible spongiform encephalopathies (TSEs): Enkephalos from the Greek for brain and pathos meaning disease. His work led to the theory that the infectious agent was not a virus as proposed by Gajdusek but was a protein which he called a prion. The story of these two Nobelists is clouded by the personalities of each awardee: Gajdusek was described as a scientific genius with the emotional maturity of a child. He would eventually end up disgraced and in exile from the U.S. Prusiner was described by his colleagues as aggressive and egotistical, and as one colleague put it, he had never seen anybody covet the Nobel as openly as Prusiner.