NEWS & VIEWS NATURE|Vol 457|22 January 2009

OBITUARY Daniel Carleton Gajdusek (1923–2008) The most outlandish and peripatetic of microbe hunters.

Daniel Carleton Gajdusek, who won the called prions, these agents are misfolded AP 1976 Nobel Prize in Physiology or Medicine proteins that can induce misfolding in for his discovery of transmissible dementias, other proteins. The initiation of misfolding died on 12 December 2008 in a hotel in falls in the twilight zone between normal Tromsø, Norway. This isolated spot above and abnormal protein production, and is the Arctic Circle was the winter refuge for still not understood. The conspicuously American-born Gajdusek during the last unconventional Gajdusek had thus found 10 years of his life. He spent his summers a suitably eccentric infectious agent. The mainly in Amsterdam. This migratory discovery won him a Nobel prize, and earned pattern, and his choice to spend winter in him the right to add his name to the staircase one of the darkest places on Earth, typified of the world’s great microbe hunters. the eccentricity of the man. Eccentricity was the source of Gajdusek’s Gajdusek was born in 1923 in Yonkers, New genius as a scientist, and of his notoriety York, where his father had a butcher’s shop. As late in life. In 1997, he was imprisoned on a an eight year old, he already seemed to know child molestation charge involving one of the his destiny. He told me once that he inscribed more than 50 Micronesian and Melanesian the names of all the scientists in Paul de Kruif’s children he had adopted and brought to book Microbe Hunters — which included Melbourne, Australia, with Frank Macfarlane the United States. On his release in 1998 he giants such as Robert Koch and Louis Pasteur Burnet, who received a Nobel prize in 1960 moved to Europe, which he regarded as less — on the staircase to his attic chemistry lab, for his work on the recognition of ‘self’ puritanical than his home country. leaving the last step blank for himself. by the immune system. His experiences Throughout his life, Gajdusek was a fervent Gajdusek’s mind was continuously in this vibrant field taught him to expect reader of the world’s literature and a prolific attracted to the mysterious and exceptional, the unexpected, and prepared him for his writer. He believed in a life of learning and in his rationale being that, to contribute to greatest discovery. accurate documentation and reflection. An knowledge, you must find unexplained In 1957, Gajdusek travelled to New example of his acuity was his letter writing. phenomena and observe them first-hand. Guinea upon hearing that Vincent Zigas, a When you wrote to him, you got your own From the early 1950s onwards, having trained district medical officer, had stumbled across letter back with an answer to each sentence as a research virologist, he recorded his a mysterious illness — kuru — in the Fore scribbled between the lines. scientific endeavours on film so that he could tribe of the Highlands. This turned out to Around two months before his death, I had share his experiences as directly as possible be a neurological disease affecting women dinner with him at the Academic Club of the with everybody. and children. It progressed swiftly from an University of Amsterdam, about a minute’s In 1954, Gajdusek shot a documentary initially unsteady gait to tremors and speech walk from his university lodgings. It took entitled Rabies in Man, which followed disorders, leading within months to complete us at least 20 minutes to get there, stopping experiments at the Pasteur Institute of Iran incapacitation, and invariably to death. every minute to rest because of his failing in Tehran. The institute’s director, Marcel Gajdusek suspected from the beginning that heart. This cumbersome trip did not stop Baltazard, had recently shown that almost the disease was caused by a form of ritual him from talking all the way about his most a third of people who had suffered a rabid- cannibalism in which only women and recent interest: the physical evidence in the dog bite to the head could not be saved by children participated. brain revealing a person’s reading ability and rabies vaccine. Baltazard considered this In 1961, Gajdusek convinced Clarence the development of that skill. He kept yelling result disastrous. Gajdusek suggested that he Joseph Gibbs Jr, a specialist in insect-borne at me, while gasping for air, that before the should test anti-rabies antibodies (prepared viruses, to lead a series of experiments age of six a child could achieve native fluency from rabbit serum by Herald Cox in New designed to establish the concept of in at least six, if not ten, languages, if properly York) in combination with the vaccine. ‘transmissible spongiform encephalopathies’ exposed. He must have had his own youth Baltazard agreed to this, and in August 1954 (TSEs). Besides kuru, TSEs include in mind: Gajdusek could read at least ten he began using the combination therapy in neurodegenerative illnesses such as languages. 18 patients who had sustained head wounds Creutzfeldt–Jakob disease (CJD) and ‘mad Gajdusek will be remembered for both his from rabid wolves. Gajdusek’s documentary cow’ disease. Gajdusek and Gibbs reported scientific contributions and his overwhelming meticulously followed their progress during the successful transmission of kuru to presence. As Richard Rhodes observed in his treatment. The study showed convincingly chimpanzees in 1966, of CJD to chimpanzees book on TSEs, Deadly Feasts, Gajdusek was that addition of rabies antibodies to the in 1968, and of scrapie (the sheep variant of “A compulsive talker who spills ideas nonstop vaccine can completely protect people from TSE) to monkeys in 1972. In later years, CJD for hours — good talk, often brilliant talk and disease or death after exposure to rabies virus. was categorized with Alzheimer’s disease as consummate story-telling, but more than This regimen has been the gold standard of an amyloidosis (diseases characterized by some listeners can bear”. care for the disease ever since. the deposition of insoluble proteins), but Jaap Goudsmit Gajdusek’s views on microbiology were Gajdusek and co-workers showed in 1980 Jaap Goudsmit is in the Research and shaped by the training in physical chemistry that Alzheimer’s disease, unlike CJD, was not Development Department of Crucell Holland, PO that he received from Linus Pauling, and transmissible. Box 2048, Leiden, 2301 CA, the Netherlands, and by schooling in cell biology and virology Gajdusek’s work revealed the existence in the Academic Medical Center of the University from John Enders — both Nobel laureates. of a new kind of infectious agent, one that of Amsterdam. From 1955 to 1957, he also worked in did not need a nucleic acid to replicate. Now e-mail: [email protected] 394 © 2009 Macmillan Publishers Limited. All rights reserved