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Daniel Carleton Gajdusek
Daniel Carleton Gajdusek 2bw.jpg
Born (1923-09-09)September 9, 1923
Died December 12, 2008(2008-12-12) (aged 85)
Nationality American
Alma mater University of Rochester, Harvard Medical School
Known for Early discovery of prion disease
Awards E. Mead Johnson Award (1963)
Nobel Prize in Physiology or Medicine (1976)
Scientific career
Fields Medicine
Influenced Lukas Plank

Daniel Carleton Gajdusek (/ˈɡdəʃɛk/ ghy--shek; 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 the transmissibility of kuru, implying the existence of an infectious agent, which he named an 'unconventional virus'.

..... His papers are held at the National Library of Medicine in Bethesda, Maryland. and at the American Philosophical Society in Philadelphia, Pennsylvania.

Early life and education

Gajdusek's father, Karol Gajdusek, was a butcher from 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. 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. From 1970 to 1996, Gajdusek was the chief of the Laboratory of Central Nervous System Studies at NINDS at the National Institutes of Health (NIH). He was elected as a member of the American Philosophical Society in 1978.

Kuru research

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. He did this by drilling holes into chimps' heads and placing pureed brain matter into the cerebellum. 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. 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"

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 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 at a low level, despite their radiation resistance.

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

Death and legacy

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.

..... The novel centers on a character named A. Norton Perina, inspired by Gajdusek, who researches the life-extending properties of sacred turtle meat in Micronesia.

Works

Books

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

Articles and monographs

Daniel C. Gajdusek was a prolific science author come diarist, and published over 1,000 original papers, reviews and commentaries 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.

See also

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