Prion pioneer dies

D. Carleton Gajdusek, a virologist and anthropologist who won the 1976 Nobel Prize for his work on the infectious brain agents now known as prions, died last Friday (Dec. 12) in Tromso, Norway. He was 85. "He was a genius," linkurl:Robert Klitzman,;http://www.cumc.columbia.edu/dept/bec/staff/klitzman.html a psychiatrist at Columbia University in New York and Gajdusek's biographer, told __The Scientist__. "His brain was faster and at a higher level than anyone I've ever met." In the 1950s, linkurl:Gajdusek;http://nobelprize.org/nobel_prizes/medicine/laureates/1976/gajdusek-autobio.html (pronounced Guy-dah-shek) began studying the linkurl:Fore people;http://en.wikipedia.org/wiki/Fore_(people) of the New Guinea highlands, a cannibalistic tribe that suffered from a neurological disorder known as "kuru," which left victims brains riddled with spongy holes. He suspected that the disease was transmitted by the Fore custom of ritualistically eating the brains of deceased ancestors, but he could not find ordinary signs of any pathogenic organism. He injected mashed brains from kuru victims into chimpanzees' brains, and when the chimps developed kuru more than a year later, he theorized that slow-acting viruses were at work. By the late 1960s, Gajdusek and his colleagues had shown that kuru was not unique, but part of a hitherfore unknown group of diseases called spongiform encephalopathies. "He turned on its head the whole [idea] that DNAs and RNAs are involved for all life," Klitzman said. Not knowing the exact source of the disease, Gajdusek called the mysterious infectious agents "slow unconventional viruses" -- quite an appropriate name, according to Klitzman. "That he would seek to name a life form 'unconventional' was very indicative of the man," he said. Gajdusek's original description of kuru, published in 1957 in the__ linkurl:New England Journal of Medicine,;http://www.ncbi.nlm.nih.gov/pubmed/13483871 __has been cited around 300 times, according to ISI. His 1966 Nature paper describing kuru transmission to chimpanzees has been cited around 500 times. linkurl:Stanley Prusiner,;http://prusinerlab.ucsf.edu/people/director.php a neurologist at University of California, San Francisco, later identified the infectious agents as tangles of rogue, misfolded proteins called prions. Prions are now recognized as the cause of kuru, human Creutzfeldt-Jakob disease, and so-called mad cow disease, as well as potentially triggering some dementias and cancers. Pruisner won his own Nobel in 1997. Gajdusek also studied isolated communities around the world to investigate the genetics of other rare diseases including pseudo-hermaphroditism and Huntington's disease. "[Gajdusek] was an outstanding and very unusual character," Lev Goldfarb, a neurologist at the NIH's National Institute of Neurological Disorders and Stroke (NINDS) in Bethesda, Maryland, told __The Scientist__. "Ordinary colleagues like me would not be able to make the far-reaching comparisons and insights that he was able to make." Gajdusek was born on September 9, 1923, in Yonkers, New York. He held a bachelor's degree from the University of Rochester, and a M.D. from Harvard Medical School. In the 1950s, he worked at the Pasteur Institute in Iran and the Walter and Eliza Hall Institute for Medical Research in Australia before joining the NIH in 1958. From 1970 to 1997, he headed up the NINDS. In 1997, Gajdusek became embroiled in controversy after he was charged with molesting several of the young boys that he had adopted while on expeditions to the Pacific. After pleading guilty to one charge and serving a year in prison, he spent the remainder of his life living in Europe. "He has a rather complex legacy," Klitzman said. "On the one hand, I don't think it detracted from what he did as a scientist. But as a man or human being or humanitarian, we have to look at all the aspects of his life." Gajdusek remained unapologetic about his sexual relationships, and asserted that sex with young men was customary in the cultures he studied, Klitzman added. "He was divided between the cultures," said Goldfarb. "What he would consider absolutely normal over there is not normal over here. He never fully accepted the realities of this culture." Although Gajdusek won his Nobel Prize for work on infectious diseases, Gajdusek himself saw his anthropological research as his most significant achievement, said Klitzman. "He [felt] that his biggest contribution was showing that you could take someone born in the Stone Age, bring them to this country... and someone could jump through 5000 years of human society and cultural advancement in one lifetime." **__Related stories:__***linkurl:Prion hypothesis proven?;http://www.the-scientist.com/article/display/22653/ [21st April 2005]*linkurl:Portals for prions?;http://www.the-scientist.com/article/display/12516/ [23rd July 2001]*linkurl:Prions' changeability;http://www.the-scientist.com/article/display/18534/ [10th May 1999]

Prion pioneer dies

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