Daniel Carleton Gajdusek

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Daniel Carleton Gajdusek
Daniel Carleton Gajdusek 2bw.jpg
Born(1923-09-09)September 9, 1923
DiedDecember 12, 2008(2008-12-12) (aged 85)
NationalityUnited States
Alma materUniversity of Rochester, Harvard Medical School
Known forEarly discovery of prion disease
AwardsE. Mead Johnson Award (1963)
Nobel Prize in Physiology or Medicine (1976)
Scientific career
FieldsMedicine
InfluencedLukas Plank

Daniel Carleton Gajdusek (/ˈɡdəʃɛk/ GHY-də-shek;[1] September 9, 1923 – December 12, 2008) was an American physician and medical researcher who was the co-recipient (with Baruch S. Blumberg) of the Nobel Prize in Physiology or Medicine in 1976 for work on an infectious agent which would later be identified as kuru, the first known human prion disease.[2]

In 1996, Gajdusek was charged with child molestation and, after being convicted, spent 12 months in prison before entering a self-imposed exile in Europe, where he died a decade later. His papers are held at the National Library of Medicine in Bethesda, Maryland.[3] and at the American Philosophical Society in Philadelphia, Pennsylvania.[4]

Early life and education[edit]

Gajdusek's father, Karol Gajdusek, was a butcher, an ethnic Slovak from Büdöskő, Kingdom of Hungary (now Smrdáky, Slovakia). His maternal grandparents, ethnic Hungarians of the Calvinist faith, emigrated from Debrecen, Hungary. Gajdusek was born in Yonkers, New York, and graduated in 1943 from the University of Rochester, where he studied physics, biology, chemistry and mathematics. He obtained an M.D. from Harvard University in 1946 and performed postdoctoral research at Columbia University, the California Institute of Technology, and Harvard. In 1951, Gajdusek was drafted into the U.S. Army and assigned as a research virologist at the Walter Reed Army Medical Service Graduate School.[5] In 1954, after his military discharge, he went to work as a visiting investigator at the Walter and Eliza Hall Institute of Medical Research in Melbourne, Australia. There, he began the work that culminated in the Nobel prize.

Kuru research[edit]

Gajdusek's best-known work focused on kuru. This disease was rampant among the South Fore people of New Guinea in the 1950s and 1960s. Gajdusek connected the spread of the disease to the practice of funerary cannibalism by the South Fore. With elimination of cannibalism, kuru disappeared among the South Fore within a generation.

Gajdusek was introduced to the problem of kuru by Vincent Zigas, a district medical officer in the Fore Tribe region of New Guinea. Gajdusek provided the first medical description of this unique neurological disorder, which was miscast in the popular press as the "laughing sickness" because some patients displayed risus sardonicus as a symptom. He lived among the Fore, studied their language and culture, and performed autopsies on kuru victims.

Gajdusek concluded that kuru was transmitted by the ritualistic consumption of the brains of deceased relatives, which was practiced by the Fore. He then proved this hypothesis by successfully transmitting the disease to primates and demonstrating that it had an unusually long incubation period of several years.[6] He did this by drilling holes into chimps' heads and placing pureed brain matter into the cerebellum.[7][8] These animals then developed symptoms of kuru. This was the first demonstration of the infectious spread of a noninflammatory degenerative disease in humans.

Kuru was shown to have remarkable similarity to scrapie, a disease of sheep and goats caused by an unconventional infectious agent. Subsequently, additional human agents belonging to the same group were discovered. They include sporadic, familial, and variant Creutzfeldt–Jakob disease. Gajdusek recognized that diseases like Kuru and Creutzfeldt–Jakob disease were caused by a new infectious agent that had not yet been identified.[2] Further research on the scrapie agent by Stanley Prusiner and others led to the identification of endogenous proteins called prions as the cause of these diseases.

"Unconventional viruses"[edit]

In his 1977 paper "Unconventional Viruses and the Origin and Disappearance of Kuru," Gajdusek postulated that the cause of kuru, scrapie and Creutzfeldt–Jakob disease were caused by what he termed an unconventional virus. In comparison to normal viruses, unconventional viruses had a long incubation period and did not cause an immune system response in the host. Although Gajdusek noted that there were no demonstrable nucleic acids in unconventional viruses, he did not rule out the possibility that unconventional viruses contained RNA in a smaller amount despite radiation resistance [9]

These infectious agents were later discovered to be misfolded proteins, or prions.

Child molestation allegations and convictions[edit]

In the course of his research trips in the South Pacific, Gajdusek had brought 56 mostly male children back to live with him in the United States, and provided them with the opportunity to receive high school and college education. One of these boys, now a grown man, later accused Gajdusek of molesting him as a child.

Gajdusek was charged with child molestation in April 1996, based on incriminating entries in his personal diary and statements from a victim. He pleaded guilty in 1997 and, under a plea bargain, was sentenced to 12 months in jail. After his release in 1998, he was permitted to serve his five-year unsupervised probation in Europe. He never returned to the United States and lived in Amsterdam, spending winters in Tromsø, Norway,[10] where the polar night around the winter solstice helped him to do more work.[1]

Gajdusek's treatment was denounced in October 1996 as anti-elitist and unduly harsh by controversial former Edinburgh University psychologist Chris Brand.[11]

The documentary The Genius and the Boys by Bosse Lindquist, first shown on BBC Four on June 1, 2009, notes that "seven men testified in confidentiality about Gajdusek having had sex with them when they were boys," that four said "the sex was untroubling" while for three of them "the sex was a shaming, abusive and a violation." One of these boys, the son of a friend and now an adult, appears in the film. Furthermore, Gajdusek openly admits to molesting boys and his approval of incest.[12] The film tries to analyse Gajdusek's sexual behaviour, and also to understand his motivations for science, exploration and life.

Death and legacy[edit]

Gajdusek died December 12, 2008, in Tromsø, Norway, at the age of 85. He was working and visiting colleagues in Tromsø at the time of his death.[13]

Hanya Yanagihara's 2013 novel, The People in the Trees, is based on Gajdusek's life, research, and child molestation conviction.[14] The novel centers on a character named A. Norton Perina, inspired by Gajdusek, whose research is conducted on the life-extending properties of turtle meat in Micronesia.[15]

Works[edit]

Books[edit]

  • Acute Infectious Hemorrhagic Fevers and Mycotoxicosis in the Union of Soviet Socialist Republics (1953), Washington, DC: Walter Reed Army Medical Center.

Articles and monographs[edit]

Daniel C. Gajdusek published hundreds of articles in scientific and medical journals. This is an incomplete list of some of the more cited ones.

  • Gajdusek, Daniel Carleton. Unconventional viruses and the origin and disappearance of kuru. National Institutes of Health, 1977.
  • Gajdusek; Carleton, Daniel; Gibbs, Clarence J.; Alpers, Mâ (1966). "Experimental transmission of a Kuru-like syndrome to chimpanzees". Nature. 209 (5025): 794–796. Bibcode:1966Natur.209..794G. doi:10.1038/209794a0. PMID 5922150. S2CID 39242676.
  • Gajdusek, Daniel Carleton (1957). "Degenerative disease of the central nervous system in New Guinea: the endemic occurrence of" kuru" in the native population". New England Journal of Medicine. 257 (20): 974–978. doi:10.1056/nejm195711142572005. PMID 13483871.
  • Gajdusek, Daniel Carleton (1985). "Hypothesis: interference with axonal transport of neurofilament as a common pathogenetic mechanism in certain diseases of the central nervous system". The New England Journal of Medicine. 312 (11): 714–719. doi:10.1056/nejm198503143121110. PMID 2579335.
  • Gibbs, Clarence J.; Carleton Gajdusek, Daniel (1969). "Infection as the etiology of spongiform encephalopathy (Creutzfeldt–Jakob disease)". Science. 165 (3897): 1023–1025. Bibcode:1969Sci...165.1023G. doi:10.1126/science.165.3897.1023. PMID 5804726. S2CID 206567023.

Further reading[edit]

External links[edit]

References[edit]

  1. ^ a b Holley, Joe (December 16, 2008) "D. Carleton Gajdusek; Controversial Scientist", The Washington Post, p. B5.
  2. ^ a b "Physiology or Medicine 1976 – Press Release". Nobelprize.org. October 14, 1976. Retrieved July 21, 2018.
  3. ^ "D. Carleton Gajdusek Papers 1918–2000". National Library of Medicine.
  4. ^ "D. Carleton (Daniel Carleton) Gajdusek correspondence, 1934-1988". American Philosophical Society.
  5. ^ Maugh, Thomas (December 18, 2008). "D. Carleton Gajdusek dies at 85; Nobel Prize winner identified exotic disease, was unrepentant pedophile". Los Angeles Times. Retrieved May 12, 2012.
  6. ^ Gajdusek, D. Carleton; Gibbs, Clarence J., Jr.; Alpers, Michael (January 13, 1967). "Transmission and passage of experimental 'kuru' to chimpanzees". Science. 155 (3759): 212–214. Bibcode:1967Sci...155..212C. doi:10.1126/science.155.3759.212 (inactive January 9, 2021). PMID 6015529.CS1 maint: DOI inactive as of January 2021 (link)
  7. ^ Kelleher, Colm A. (2004). Brain Trust: The Hidden Connection Between Mad Cow and Misdiagnosed Alzheimer's Disease. Simon & Schuster. p. 53. ISBN 9781416507567.
  8. ^ "Fatal Infections". New England Anti-Vivisection. 2013. Retrieved July 23, 2013.
  9. ^ Gajdusek, D. Carleton (1977). "Unconventional Viruses and the Origin and Disappearance of Kuru". Science. 197 (4307): 943–960. Bibcode:1977Sci...197..943C. doi:10.1126/science.142303. JSTOR 1744284. PMID 142303. S2CID 2046908.
  10. ^ Richmond C (February 24, 2009). "Carleton Gajdusek". The Guardian. Retrieved May 9, 2019.
  11. ^ Wojtas, Olga (March 27, 1998). "'Racist' Brand loses dismissal appeal". Times Higher Education. Retrieved May 12, 2012.
  12. ^ Lindquist, Bosse (June 1, 2009). "The Genius and the Boys". Storyville. BBC Four. Retrieved May 12, 2012.
  13. ^ McNeil, Donald G., Jr. (December 15, 2008). "D. Carleton Gajdusek, Who Won Nobel for Work on Brain Disease, Is Dead at 85". The New York Times. Retrieved May 12, 2012.
  14. ^ Ciuraru, Carmela (September 27, 2013). "Bitter Fruit: 'The People in the Trees,' by Hanya Yanagihara". The New York Times. Retrieved March 12, 2015.
  15. ^ Yanagihara, Hanya (August 13, 2013). The People in the Trees. Doubleday. ISBN 978-0385536776.