Kuru, the first human transmissible spongiform encephalopathy was transmitted to chimpanzees by D. Carleton Gajdusek (1923–2008). In this review, I briefly summarize the history of this seminal discovery along its epidemiology, clinical picture, neuropathology and molecular genetics. The discovery of kuru opened new windows into the realms of human medicine and was instrumental in the later transmission of Creutzfeldt-Jakob disease and Gerstmann-Str？ussler-Scheinker disease as well as the relevance that bovine spongiform encephalopathy had for transmission to humans. The transmission of kuru was one of the greatest contributions to biomedical sciences of the 20th century.
References
[1]
Asher, D.M.; Gibbs, C.J., Jr.; Sulima, M.P.; Bacote, A.; Amyx, H.; Gajdusek, D.C. Transmission of Human Spongiform Encephalopathies to Experimental Animals: Comparison of Chimpanzee and Squirrel Monkey. In Transmissible Spongiform Encephalopathies – Impact on Animal and Human Health; Brown, P., Ed.; Developments of Biological Standardization, Karger: Basel, Switzerland, 1993; Volume 80, pp. 9–13.
[2]
Asher, D.M. Kuru: Memories of the NIH years. Phil. Trans. R. Soc. B 2008, 363, 3618–3625, doi:10.1098/rstb.2008.4002.
[3]
Gajdusek, D.C. Kuru: An appraisal of five years of investigation. Eugen. Q. 1962, 9, 69–74.
[4]
Gibbs, C.J., Jr.; Gajdusek, D.C. Infection as the etiology of spongiform encephalopathy (Creutzfeldt-jakob disease). Science 1969, 165, 1023–1025.
[5]
Hornabrook, R.W. Kuru. Papua New Guinea Med. J. 1975, 4, 203–206.
[6]
Hornabrook, R.W. Kuru and clinical neurology. In Slow Transmissible Diseases of the Nervous System; Prusiner, S.B., Hadlow, W.J., Eds.; Academic Press: New York, NY, USA, 1979; pp. 37–66.
[7]
Liberski, P.P.; Brown, P. Kuru ？ fifty years later. Neurol. Neurochir. Pol. 2007, 41, 548–556.
[8]
Liberski, P.P.; Brown, P. Kuru: A half-opened window onto the landscape of neurodegenerative diseases. Folia Neuropathol. 2004, 42 Suppl A, 3–14.
[9]
Liberski, P.P.; Brown, P. Kuru: Its ramifications after fifty years. Exp. Gerontol. 2009, 44, 63–69, doi:10.1016/j.exger.2008.05.010.
[10]
Liberski, P.P.; Brown, P. Prion Disease: From Ritualistic Endocannibalism to Cellular Endocannibalism – From Kuru to Autophagy. In Aging and Age-Related Disease: The Basics; Karasek, M., Ed.; Nova Science Publisher, Inc.: New York, NY, USA, 2006; pp. 373–420.
[11]
Liberski, P.P.; Gajdusek, D.C. Kuru: Fourty years later, a historical note. Brain Pathol. 1997, 7, 555–560, doi:10.1111/j.1750-3639.1997.tb01073.x.
[12]
Gajdusek, D.C.; Zigas, V. Clinical, pathological and epidemiological study of an acute progressive degenerative disease of the central nervous system among natives of the Eastern Highlands of New Papua. Am. J. Med. 1959, 26, 442–469, doi:10.1016/0002-9343(59)90251-7.
[13]
Gajdusek, D.C.; Zigas, V. Studies of kuru. I. The ethnologic setting of kuru. Am. J. Trop. Med. Hyg. 1961, 10, 80–91.
[14]
Gajdusek, D.C.; Gibbs, C.J., Jr.; Alpers, M. Transmission and passage of experimental kuru to chimpanzees. Science 1967, 155, 212–214.
[15]
Gajdusek, D.C.; Gibbs, C.J., Jr. Transmission of two subacute spongiform encephalopathies of man (kuru and Creutzfeldt-Jakob disease) to New World monkeys. Nature 1971, 230, 588–591, doi:10.1038/230588a0.
[16]
Gajdusek, D.C.; Gibbs, C.J.; Alpers, M. Experimental transmission of a kuru-like syndrome to chimpanzees. Nature 1966, 209, 794–796, doi:10.1038/209794a0.
[17]
Gajdusek, D.C.; Rogers, N.G.; Basnight, M.; Gibbs, C.J., Jr.; Alpers , M.P. Transmission experiments with kuru in chimpanzees and the isolation of latent viruses from the explanted tissues of affected animals. Ann. N. Y. Acad. Sci. 1969, 162, 529–550, doi:10.1111/j.1749-6632.1969.tb56401.x.
[18]
Gajdusek, D.C.; Zigas, V. Degenerative disease of the central nervous system in New Guinea. The endemic occurrence of “kuru” in the native population. N. Engl. J. Med. 1957, 257, 974–978, doi:10.1056/NEJM195711142572005.
Jaskolski, M.; Liberski, P.P. Kurt Wüthrich – co-winner of the Nobel Prize in Chemistry, 2002. Acta Neurobiol. Exp. 2002, 62, 288–289.
[21]
Gajdusek, D.C. Kuru and its contribution to medicine. Phil. Trans. R. Soc. 2008, 363, 3697–3700, doi:10.1098/rstb.2008.0070.
[22]
Beyreuther, K.; Masters, C.L. βA4-Amyloid Domain is Essential for Axonal Sorting of APP: Implications for Alzheimer’s Disease. In Proceedings of Abstracts of the satellite meeting “Brain Tumors and Alzheimer’s Disease, From Neuropathology to Molecular Biology”, Bali, Indonesia, 3–5 September 1997.
[23]
Gajdusek, D.C. Infectious Amyloids: Subacute Spongiform Encephalopathies as Transmissible Cerebral Amyloidosis. In Fields Virology, 3rd Ed.; Fields, B.N., Knippe, D.M., Howley, P.M., Eds.; Lippincott-Raven Publ: Philadelphia, PA, USA, 1996; pp. 2851–2900.
[24]
Gajdusek, D.C. Molecular Casting of Infectious Amyloids, Inorganic and Organic Replication: Nucleation, Conformational Change and Self-Assembly. In Self-Assembling Peptide Systems in Biology, Medicine and Engineering; Aggeli, A., Boden, N., Zhang, S., Eds.; Springer: New York, 2001; pp. 105–112.
[25]
Ashe, K.H.; Aguzzi, A. Prions, prionoids and pathogenic proteins in Alzheimer disease. Prion 2013, 7, 55–59, doi:10.4161/pri.23061.
[26]
Gajdusek, D.C.; Alpers, M.P.; Gibbs, C.J., Jr. Kuru: Epidemiological and Virological Studies of Unique New Guinean Disease with Wide Significance to General Medicine. In Essays on Kuru; Hornabrook, R.W., Ed.; E.W. Classey Ltd.: Faringdon, Berks, UK, 1976; pp. 125–145.
[27]
Gajdusek, D.C.; Alpers, M.P. Genetic studies in relation to kuru. I. Cultural, historical, and demographic background. Am. J. Hum. Gen. 1972, 24, 1–38.
[28]
Gajdusek, D.C.; Alpers, M.P. Kuru in childhood: Disappearance of the disease in the younger age group7th Annual Meeting of American Pediatric Society. J. Pediatr. 1966, 69, 886–887, doi:10.1016/S0022-3476(66)80483-3.
[29]
Kuru. Early Letters and Field-Notes from the Collection of D. Carleton Gajdusek; Gajdusek, D.C., Farquhar, J., Eds.; Raven Press: New York, NY, USA, 1981.
[30]
Gajdusek, D.C.; Gibbs, C.J. Unconventional Viruses Causing the Spongiform Virus Encephalopathies. A Fruitless Search for the Coat and Core. In Viruses and Environment; Kurstak, E., Maramosch, K., Eds.; Academic Press: New York, NY, USA, 1978; pp. 79–98.
[31]
Gajdusek, D.C.; Reid, H.L. Studies of kuru. IV: The kuru pattern in Moke, a representative Fore village. Am. J. Trop. Med. Hyg. 1961, 10, 628–638.
[32]
Gajdusek, D.C. Early images of kuru and the people of Okapa. Phil. Trans. R. Soc. 2008, 363, 3636–3643, doi:10.1098/rstb.2008.4011.
[33]
Gajdusek, D.C. Kuru in the New Guinea Highlands. In Tropical Neurology; Spillane, J., Ed.; Oxford University Press: London, UK, 1973; pp. 376–383.
[34]
Gajdusek, D.C. Le kuru. In Colloque sur les Viruses lents, a Talloires, France, Organize le 17 Septembre 1978; pp. 25–57. Collection Foundation Merieux, 1979.
[35]
Gajdusek, D.C. Observations on the Early History of Kuru Investigation. In Slow Transmissible Diseases of the Nervous System; Prusiner, S.B., Hadlow, W.J., Eds.; Academic Press: New York, NY, USA, 1979; Volume 1, pp. 7–36.
[36]
Gajdusek, D.C. Slow virus infections of the nervous system. N. Engl. J. Med. 1967, 276, 392–400, doi:10.1056/NEJM196702162760708.
[37]
Gajdusek, D.C. Unconventional Viruses and the Origin and Disappearance of Kuru. In Les Prix Nobel en 1976; Nobel Fdn PA Norstedt & Soner: Stockholm, Sweden, 1977; pp. 167–216.
[38]
Gajdusek, D.C. Unconventional viruses and the origin and disappearance of kuru. Science 1977, 197, 943–960.
[39]
Gajdusek, D.C. Urgent Opportunistic Observations: The study of Changing, Transient and Disappearing Phenomena of Medical Interst in Disrupted Primitive Human Communities. In Health and Disease in Tribal Societies; CIBA Foundation Symposium, Elsevier: Amsterdam, The Netherlands, 1977; pp. 69–102.
[40]
Zigas, V.; Gajdusek, D.C. Kuru. Clinical study of a new syndrome resembling paralysis agitans in natives of the eastern Highlands of Australian New Guinea. Med. J. Aust. 1959, 44, 745–754.
[41]
Zigas, V.; Gajdusek, D.C. Kuru. Clinical, pathological and epidemiological study of a recently discovered acute progressive degenerative disease of the central nervous system reaching “epidemic” proportions among natives of the Eastern Highlands of New Guinea. Papua New Guinea Med. J. 1959, 3, 1–31.
[42]
Mathews, J.D. The changing face of kuru: A personal perspective. Phil. Trans. R. Soc. 2008, 363, 3679–3684, doi:10.1098/rstb.2008.0085.
[43]
Mathews, J.D. The epidemiology of kuru. Papua New Guinea Med. J. 1967, 10, 76–82.
[44]
Lindenbaum, S. Cannibalism, kuru and anthropology. Folia Neuropathol. 2009, 47, 138–144.
[45]
Lindenbaum, S. Kuru Sorcery. Disease and Danger in the New Guinea Highlands, 2nd Ed. ed.; Paradigm Publishers: Colorado, CO, USA, 2013; p. 224.
[46]
Bennett, J.H.; Rhodes, F.A.; Robson, H.N. A possible genetic basis for kuru. Am. J. Hum. Gen. 1959, 11, 169–187.
[47]
Bennett, J.H.; Rhodes, F.A.; Robson, H.N. Observations on kuru. I. A possible genetic basis. Australas Ann. Med. 1958, 7, 269–275.
[48]
Glasse, R.; Lindenbaum, S. Fieldwork in the South Fore: The Process of Ethnographic Inquiry. In Prion Diseases of Humans and Animals; Prusiner, S.B., Collinge, J., Powell, J., Anderton, B., Eds.; Ellis Horwood: New York, London, Toronto, Sydney, Singapore, 1993; pp. 77–91.
[49]
Lindenbaum, S. Understanding kuru: The contribution of anthropology and medicine. Phil. Trans. R. Soc. 2008, 363, 3715–3720, doi:10.1098/rstb.2008.0072.
[50]
Lindenbaum, S. Images of Catastrophe: The Making of an Epidemic. In The Political Economy of AIDS; Singer, M., Ed.; Baywood Publ Co Inc.: Amityville, New York, NY, USA, 1998; pp. 33–58.
[51]
Lindenbaum, S. Kuru, prions, and human affairs. Ann. Rev. Antrop. 2001, 30, 363–385, doi:10.1146/annurev.anthro.30.1.363.
[52]
Lindenbaum, S. Thinking about cannibalism. Ann. Rev. Antrop. 2004, 33, 475–498, doi:10.1146/annurev.anthro.33.070203.143758.
[53]
Matthews, J.D.; Glasse, R.M.; Lindenbaum, S. Kuru and cannibalism. Lancet 1968, 2, 449–452.
[54]
Glasse, R. Cannibalism in the Kuru region of New Guinea. Trans. N. Y. Acad. Sci. 1967, 29, 748–754, doi:10.1111/j.2164-0947.1967.tb02298.x.
[55]
Arens, W. The Man-Eating Myth: Anthropology and Anthropophagy: Anthropology and Anthropophagy; Oxford University Press: New York, NY, USA, 1979.
[56]
Zigas, V. Origin of Investigations on Slow Virus Infections in Man. In Slow Transmissible Diseases of the Nervous System; Prusiner, S.B., Hadlow, W.J., Eds.; Academic Press: New York, NY, USA, 1979; Volume 1, pp. 3–6.
[57]
Hamilton, L.; Gajdusek, D.C. Nutrition in the kuru region. II. A nutritional evaluation of tradition Fore diet in Moke village in 1957. Acta Trop. 1969, 26, 331–345.
[58]
Sorenson, E.R.; Gajdusek, D.C. Nutrition in the kuru region. I. Gardening, Food handling, and diet of the Fore people. Acta Trop. 1969, 26, 281–330.
[59]
Sorenson, E.R.; Gajdusek, D.C. The study of child growth and development in primitive cultures. A research archive for ethnopediatric film investigations of styles in the patterning of the nervous system. Pediatrics 1966, 37, 149–243.
[60]
Curtain, C.C.; Gajdusek, D.C.; Zigas, V. Studies on kuru. II. Serum proteins in natives from the kuru region of New Guinea. Am. J. Trop. Med. Hyg. 1961, 10, 92–109.
[61]
Kitchin, D.; Bearn, A.G.; Alpers, M.P.; Gajdusek, D.C. Genetic studies in relation to kuru. III. Distribution of the inherited serum group-specific protein (Gc) phenotypes in New Guineans: An association of kuru and the Gc Ab phenotype. Am. J. Hum. Gen. 1972, 24, S72–S85.
[62]
Mbaginta’o, I.G. Medical practices and funeral ceremony of the Dunkwi Anga. J. Soc. Océanistes 1976, 32, 299–305, doi:10.3406/jso.1976.2756.
[63]
Plato, C.C.; Gajdusek, D.C. Genetic studies in relation to kuru. IV. Dermatoglyphics of the Fore and Anga populations of the Eastern Highlands of New Guinea. Am. J. Hum. Gen. 1972, 24, S86–S93.
[64]
Simmons, R.T.; Gajdusek, D.C. Blood Group Genetical Studies on Kuru-Afflicted Natives of the Eastern Highlands of New Guimea, and Comparison with Unaffected Neighboring Tribes in Papua New Guinea. In Proceedings of the 8th Congress of International Society of Blood Transfusions, Tokyo, Japan, 12–15 September 1960; pp. 255–259.
[65]
Simmons, R.T.; Graydon, J.J.; Gajdusek, D.C.; Alpers, M.P.; Hornabrook, R.W. Genetic studies in relation to kuru. II. Blood-group genetic patterns and populations of the eastern Highlands of New Guinea. Am. J. Hum. Gen. 1972, 24, S39–S71.
[66]
Simmons, R.T.; Graydon, J.J.; Zigas, V.; Baker, V.; Larkin, L.; Gajdusek, D.C. Studies on kuru. V. A blood group genetical survey of the kuru region and other parts of Papua New Guinea. Am. J. Trop. Med. Hyg. 1961, 10, 639–664.
[67]
Simmons, R.T.; Graydon, J.J.; Zigas, V.; Baker, V.; Larkin, L.; Gajdusek, D.C. Studies on kuru. VI. Blood groups in kuru. Am. J. Trop. Med. Hyg. 1961, 10, 665–668.
[68]
Wiesenfeld, S.L.; Gajdusek, D.C. Genetic studies in relation to kuru. VI. Evaluation of increased liability to kuru in Gc, Ab-Ab indyviduals. Am. J. Hum. Gen. 1975, 27, 498–504.
[69]
Gajdusek, D.C. Kuru and Scrapie. In Prion Diseases of Humans and Animals; Prusiner, S.B., Collinge, J., Powell, J., Anderton, B., Eds.; Ellis Horwood: New York, London, Toronto, Sydney, Singapore, 1993; pp. 47–52.
[70]
Hadlow, W.J. Kuru likened to scrapie: The story remembered. Phil. Trans. R. Soc. 2008, 363, 3644, doi:10.1098/rstb.2008.4013.
[71]
Hadlow, W.J. Neuropathology and the scrapie-kuru connection. Brain Pathol. 1995, 5, 27–31, doi:10.1111/j.1750-3639.1995.tb00574.x.
[72]
Hadlow, W.J. Scrapie and kuru. Lancet 1959, 2, 289–290, doi:10.1016/S0140-6736(59)92081-1.
[73]
Hadlow, W.J. The Scrapie-Kuru Connection: Recollections of How it Came About. In Prion Diseases of Humans and Animals; Prusiner, S.B., Collinge, J., Powell, J., Anderton, B., Eds.; Ellis Horwood: New York, NY, USA, 1993; pp. 40–46.
[74]
Collinge, C.J.; Whitfield, J.; McKintosch, E.; Frosh, A.; Mead, S.; Hill, A.F.; Brandner, S.; Thomas, D.; Alpers, M.P. A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea. Phil. Trans. R. Soc. 2008, 363, 3725–3739, doi:10.1098/rstb.2008.0068.
[75]
Besnoit, C. La tremblante ou nevrite peripherique enzootique du mouton. Rev Vet (Tolouse) 1899, 24, 265–277.
[76]
M'Gowan, J.P. Investigation into the disease of sheep called “scrapie” (Traberkrankheit, La tremblante). With especial reference to its association with Sarcosporidiosis; William Blackwood and Sons: Edinburgh, Scotland, 1914.
[77]
Innes, J.R.M.; Saunders, L.Z. Comparative Neuropathology; Academic Press: New York, NY, USA, 1962; p. 839.
[78]
Goldfarb, L.G.; Cervenakova, L.; Gajdusek, D.C. Genetic studies in relation to kuru: An overview. Curr. Mol. Med. 2004, 4, 375–384, doi:10.2174/1566524043360627.
[79]
Zlotnik, I. Significance of vacuolated neurones in the medulla of sheep infected with scrapie. Nature 1957, 180, 393–394, doi:10.1038/180393a0.
[80]
Zlotnik, I. Vacuolated neurons in sheep affected with scrapie. Nature 1957, 179, 737, doi:10.1038/179737a0.
[81]
Gajdusek, D.C.; Gibbs, C.J. Attempts to demonstrate a transmissible agent in kuru, amyotrophic lateral sclerosis, and other sub-acute and chronic system degenerations of man. Nature 1964, 204, 257–259, doi:10.1038/204257a0.
Gajdusek, D.C. Transmissible and nontransmissible dementias: Distinction between primary cause and pathogenetic mechanisms in Alzheimer's disease and aging. Mount. Sinai J. Med. 1988, 55, 3–5.
[84]
Gibbs, C.J., Jr.; Gajdusek, D.C. Attempts to Demonstrate A Transmissible Agent in Kuru, Amyotrophic Lateral Sclerosis, and Other Subacute and Chronic Progressive Nervous System Degenerations of Man. In Slow, Latent, and Temperate Virus Infections; Gajdusek, D.C., Gibbs, C.J., Jr., Alpers, M., Eds.; US Department of Heath, Education, and Welfare: Washington D.C., USA, 1965; pp. 39–48.
[85]
Alpers, M.P.; Gajdusek, D.C. Changing pattern of kuru: Epidemiological changes in the period of increasing contact of the Fore people with western civilization. Am. J. Trop. Med. Hyg. 1965, 14, 852–879.
[86]
Alpers, M.P. Epidemiology and Ecology of Kuru. In Slow Transmissible Diseases of the Nervous System; Prusiner, S.B., Hadlow, W.J., Eds.; Academic Press: New York, NY, USA, 1979; Volume 1, pp. 67–90.
[87]
Alpers, M.P. The epidemiology of kuru: Monitoring the epidemic from its peak to the end. Phil. Trans. R. Soc. 2008, 363, 3707–3713, doi:10.1098/rstb.2008.0071.
[88]
Gajdusek, D.C. Kuru in Childhood: Implications for the Problem of Whether Bovine Spongiform Encephalopathy Affects Humans. In Transmissible Subacute Spongiform Encephalopathies: Prion Diseases; Court, L., Dodet, B., Eds.; Elsevier: Amsterdam, The Netherlands, 1996; pp. 15–26.
[89]
Goldfarb, L.G. Kuru: The old epidemic in a new mirror. Microbes Infect. 2002, 4, 875–882, doi:10.1016/S1286-4579(02)01608-8.
[90]
Gajdusek, D.C.; Zigas, V.; Baker, J. Studies on kuru. III. Patterns of kuru incidence: Demographic and geographic epidemiological analysis. Am. J. Trop. Med. Hyg. 1961, 10, 99–627.
[91]
Mathews, J.D. A transmission model for kuru. Lancet 1967, 1, 821–825, doi:10.1016/S0140-6736(67)92784-5.
[92]
Matthews, J.D. Kuru as An Epidemic Disease. In Essays on kuru; Hornabrook, R.W., Ed.; Papua New Guinea Institute of Medical Research, Monographs no 3: Faringdon, Berkshire, UK, 1976; pp. 83–104.
[93]
Alpers, M.P. Some tributes to research colleagues and other contributors to our knowledge about kuru. Phil. Trans. R. Soc. 2008, 363, 3614–3617, doi:10.1098/rstb.2008.4000.
[94]
Klitzman, R.L.; Alpers, M.P.; Gajdusek, D.C. The natural incubation period of kuru and the episodes of transmission in three clusters of patients. Neuroepidemiology 1984, 3, 3–20, doi:10.1159/000110837.
[95]
Cervenakova, L.; Goldfarb, L.G.; Garruto, R.; Lee, H.S.; Gajdusek, D.C.; Brown, P. Phenotype-genotype studies in kuru: Implications for new variant Creutzfeldt-Jakob disease. Proc. Natl. Acad. Sci. USA 1998, 95, 13239–13241, doi:10.1073/pnas.95.22.13239.
[96]
Brown, P.; Gibbs, C.J.; Rodgers-Johnson, P.; Asher, D.; Sulima, M.P.; Bacote, A.; Goldfarb, L.G.; Gajdusek, D.C. Human spongiform encephalopathy: The National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann. Neurol. 1994, 35, 513–529, doi:10.1002/ana.410350504.
[97]
Gibbs, C.J., Jr.; Amyx, H.L.; Bacote, A.; Masters, C.L.; Gajdusek, D.C. Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates. J. Infect. Dis. 1980, 142, 205–208, doi:10.1093/infdis/142.2.205.
[98]
Gibbs, C.J., Jr.; Gajdusek, D.C.; Amyx, H. Strain Variation in the Viruses of Creutzfeldt-Jakob Disease and Kuru. In Slow Transmissible Diseases of the Nervous System; Prusiner, S.B., Hadlow, W.J., Eds.; Academic Press: New York, NY, USA, 1979; Volume 1, pp. 87–110.
[99]
Gibbs, C.J., Jr.; Gajdusek, D.C. Transmission and Characterization of the Agents of Spongiform Virus Encephalopathies: Kuru, Creutzfeldt-Jakob Disease, Scrapie and Mink Encephalopathy. Res. Publ. Assoc. Res. Nerv. Ment. Dis. 1971, 49, 383–410.
[100]
Gibbs, C.J. Spongiform Encephalopathies – Slow, Latent, and Temperate Virus Infections – in Retrospect. In Prion Diseases of Humans and Animals; Prusiner, S.B., Collinge, J., Powell, J., Anderon, B., Eds.; Ellis Horwood: New York, London, Toronto, Sydney, Singapore, 1993; pp. 77–91.
[101]
Liberski, P.P. Wirusy powolne uk？adu nerwowego cz？owieka i zwierz？t. Cz？？？ I. Kuru. (Slow viruses of the nervous system of man and animals. part I. Kuru). Post. Hig. Med. Do？w. 1981, 35, 471–493.
[102]
Gajdusek, D.C.; Gibbs, C.J., Jr. Transmission of kuru from man to Rhesus monkeys (Macaca mulatto) 8.1/2 years after inoculation. Nature 1972, 240, 351, doi:10.1038/240351a0.
[103]
Peterson, D.A.; Wolfe, L.G.; Deinhardt, F.; Gajdusek, D.C.; Gibbs, C.J. Transmission of kuru and Creutzfeldt-Jakob disease to Marmoset monkeys. Intervirology 1974, 2, 14–19.
[104]
Masters, C.L.; Alpers, M.P.; Gajdusek, D.C.; Gibbs, C.J., Jr.; Kakulas, B.A. Experimental kuru in the gibbon and Sooty mangabey and Creutzfeldt-Jakob disease in the pigtailed macaque. J. Med. Primatol. 1976, 5, 205–209.
[105]
Beck, E.; Daniel, P.M. Kuru and Creutzfeldt-Jakob Disease: Neuropathological Lesions and Their Significance. In Slow Transmissible Diseases of the Nervous System; Prusiner, S.B., Hadlow, W.J., Eds.; Academic Press: New York, NY, USA, 1979; Volume 1, pp. 253–270.
[106]
Beck, E.; Daniel, P.M.; Alpers, M.P.; Gajdusek, D.C.; Gibbs, C.J., Jr. Neuropathological Comparisons of Experimental Kuru in Chimpanzees with Human Kuru. With a Note on Its Relation to Scrapie and Spongiform Encephalopathy. Locarno, Switzerland, 31 May —3 June 1967; Burdzy, K., Kallos, P., Eds.; Additamentum to Arch Allergy and Appl Immunol: Basel, Karger, Switzerland, 1969; Volume 36, pp. 553–562.
[107]
Beck, E.; Daniel, P.M.; Alpers, M.P.; Gajdusek, D.C.; Gibbs, C.J., Jr. Experimental kuru in chimpanzees. A pathological report. Lancet 1966, 2, 1056–1059, doi:10.1016/S0140-6736(66)92031-9.
[108]
Beck, E.; Daniel, P.M.; Asher, M.P.; Gajdusek, D.C.; Gibbs, C.J., Jr. Experimental kuru in chimpanzees. A neuropathological study. Brain 1973, 96, 441–462, doi:10.1093/brain/96.3.441.
[109]
Beck, E.; Daniel, P.M.; Gajdusek, D.C. A Comparison between the Neuropathological Changes in Kuru and Scrapie, System Degeneration. In Proceedings of the VIth International Congress Neuropathological, Zurich, Switzerland, 31 August – 3 September 1965; pp. 213–218.
[110]
Beck, E.; Daniel, P.M. Kuru and Scrapie Compared: Are They Examples of System Degeneration? In Slow, Latent, and Temperate Virus Infections; Gajdusek, D.C., Gibbs, C.J., Jr., Alpers, M.P., Eds.; US Dept Health, Education, Welfare: Washington, DC, USA, 1965; pp. 85–93.
[111]
Beck, E.; Daniel, P.M. Prion Diseases from A Neuropathologist’s Perspective. In Prion Diseases of Humans and Animals; Prusiner, S.B., Collinge, J., Powell, J., Anderton, B., Eds.; Ellis Horwood: New York, London, Toronto, Sydney, Singapore, 1993; pp. 63–65.
[112]
Beck, E.; Bak, J.; Christ, J.F.; Gajdusek, D.C.; Gibbs, C.J., Jr.; Hassler, R. Experimental kuru in the Spider monkey. Histopathological and Ultrastructural studies of the brain during early stages of incubation. Brain 1975, 98, 595–612, doi:10.1093/brain/98.4.595.
[113]
Beasley, A. The promised medicine: Fore reflections on the scientific investigation of kuru. Oceania 2006, 76, 186–202.
[114]
Alpers, M.P. Kuru: Age and Duration Studies. Mimeographed; Departament of Medicine, University of Adelaide: Adelaide, Australia, 1964; p. 12.
[115]
Alpers, M.P. Kuru: Implications of Its Transmissibility for the Interpretation of Its Changing Epidemiologic Pattern. In The Central Nervous System; Bailey, O.T., Smith, D.E., Eds.; International Academy of Pathology Monograph No. 9. Williams & Wilkins Comp: Baltimore, MD, USA, 1968; pp. 234–250.
[116]
Beasley, A. Frontier journals. Fore experiences on the kuru patrols. Oceania 2009, 79, 34–52, doi:10.1002/j.1834-4461.2009.tb00049.x.
[117]
Beasley, A. Frontier Science: The Early Investigation of Kuru in Papua New Guinea. In Challenging Science: Issues in New Zealand; Dew, K., Fitzgerald, R., Eds.; Dunmore Press: Palmerston North, New Zealand, 2004; pp. 146–166.
[118]
Beasley, A. Kuru truths: Obtaining Fore narratives. Field Methods 2006, 18, 21–42, doi:10.1177/1525822X05280177.
[119]
Beasley, A. Richard Hornabrook’s first impressions of kuru and Okapa. Phil. Trans. R. Soc. 2008, 363, 3626–3627.
[120]
Collinge, C.J.; Whitfield, J.; McKintosch, E.; Beck, J.; Mead, S.; Thomas, D.; Alpers, M.P. Kuru in the 21st century – an acquired human prion disease with very long incubation periods. Lancet 2006, 367, 2068–2074, doi:10.1016/S0140-6736(06)68930-7.
[121]
Collinge, J. Lessons of kuru research: Background to recent studies with some personal reflections. Phil. Trans. R. Soc. 2008, 363, 3689–3696, doi:10.1098/rstb.2008.0121.
[122]
Gajdusek, D.C.; Sorenson, E.R.; Meyer, J. A comprehensive cinema record of disappearing kuru. Brain 1970, 93, 65–76, doi:10.1093/brain/93.1.65.
[123]
Prusiner, S.B.; Gajdusek, D.C.; Alpers, M.P. Kuru with incubation periods exceeding two decades. Ann. Neurol. 1982, 12, 1–9, doi:10.1002/ana.410120102.
[124]
Alpers, M.P. Kuru: A Clinical Study. Mimeographed; Dept. Medicine, University of Adelaide: Adelaide, Australia, 1964; pp. 1–38.
[125]
Klatzo, I.; Gajusek, D.C.; Zigas, V. Evaluation of Pathological Findings in Twelve Cases of Kuru. In Encephalitides; van Boagert, L., Radermecker, J., Hozay, J., Lowenthal, A., Eds.; Elsevier Publ. Comp.: Amsterdam, The Netherlands, 1959; pp. 172–190.
Biernat, W.; Liberski, P.P.; Guiroy, D.C.; Yanagihara, R.; Gajdusek, D.C. Proliferating cell nuclear antigen immunohistochemistry in astrocytes in experimental Creutzfeldt-Jakob disease and in human kuru, Creutzfeldt-Jakob disease and Gerstmann-Str？ussler-Scheinker syndrome. Neurodegeneration 1995, 4, 195–201, doi:10.1006/neur.1995.0024.
[128]
Fowler, M.; Robertson, E.G. Observations on kuru. III: Pathological features in five cases. Australas Ann. Med. 1959, 8, 16–26.
[129]
Kakulas, B.A.; Lecours, A.-R.; Gajdusek, D.C. Further observations on the pathology of kuru. J. Neuropathol. Exp. Neurol. 1967, 26, 85–97, doi:10.1097/00005072-196701000-00007.
[130]
Piccardo, P.; Safar, J.; Ceroni, M.; Gajdusek, D.C.; Gibbs, C.J. Immunohistochemical localization of prion protein in spongiform encephalopathies and normal brain tissue. Neurology 1990, 40, 518–522, doi:10.1212/WNL.40.3_Part_1.518.
[131]
Scrimgeour, E.M.; Masters, C.L.; Alpers, M.P.; Kaven, J.; Gajdusek, D.C. A clinico-pathological study of case of kuru. J. Neurol. Sci. 1983, 59, 265–275, doi:10.1016/0022-510X(83)90044-8.
[132]
Scrimgeou, E.M. Some recollections about kuru in a patient at Rabaul in 1978, and subsequent experiences with prion diseases. Phil. Trans. R. Soc. 2008, 363, 3663–3664, doi:10.1098/rstb.2008.4029.
[133]
Sikorska, B.; Liberski, P.P.; Sobów, T.; Budka, H.; Ironside, J.W. Ultrastructural study of florid plaques in variant Creutzfeldt-Jakob disease: A comparison with amyloid plaques in kuru, sporadic Creutzfeldt-Jakob disease and Gerstmann-Str？ussler-Scheinker disease. Neuropathol. Appl. Neurobiol. 2009, 35, 46–59, doi:10.1111/j.1365-2990.2008.00959.x.
[134]
Seitelberger, F. Eigenartige familiar-hereditare Krankheit des Zetralnervensystems in einer niederosterreichischen Sippe. Wien Klein Wochen 1962, 74, 687–691.
[135]
Neuman, M.A.; Gajdusek, D.C.; Zigas, V. Neuropathologic findings in exotic neurologic disorder among natives of the Highlands of New Guinea. J. Neuropathol. Exp. Neurol. 1964, 23, 486–507, doi:10.1097/00005072-196407000-00007.
[136]
Masters, C.L.; Gajdusek, D.C.; Gibbs, C.J., Jr. Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Str？ussler syndrome. With an analysis of the various forms of amyloid plaque deposition in the virus induced spongiform encephalopathies. Brain 1981, 104, 559–588, doi:10.1093/brain/104.3.559.
[137]
Liberski, P.P.; Sikorska, B.; Lindenbaum, S.; Goldfarb, L.G.; McLean, C.; Hainfellner, J.A.; Brown, P. Kuru: Genes, cannibals and neuropathology. J. Neuropathol. Exp. Neurol. 2012, 71, 92–103, doi:10.1097/NEN.0b013e3182444efd.
[138]
Hainfellner, J.; Liberski, P.P.; Guiroy, D.C.; Cervénaková, L.; Brown, P.; Gajdusek , D.C.; Budka, H. Pathology and immunohistochemistry of a kuru brain. Brain Pathol. 1997, 7, 547–554, doi:10.1111/j.1750-3639.1997.tb01072.x.
[139]
McLean, C.A. The neuropathology of kuru and variant Creutzfeldt-Jakob disease. Phil. Trans. R. Soc. 2008, 363, 3685–3687, doi:10.1098/rstb.2008.0086.
[140]
McLean, C.A.; Ironside, J.W.; Alpers, M.P.; Brown, P.W.; Cervenakova, L.; Anderson, R.M.; Masters, C.L. Comparative neuropathology of kuru with the new variant of Creutzfeldt-Jakob disease: Evidence for strain of agent predominating over genotype of host. Brain Pathol. 1998, 8, 428–437.
[141]
Peat, A.; Field, E.J. An unusual structure in kuru brain. Acta Neuropathol. (Berl) 1970, 15, 288–292, doi:10.1007/BF00686775.
[142]
Liberski, P.P. The occurrence of cytoplasmic lamellar bodies in scrapie infected and normal hamster brains. Neuropatol. Pol. 1988, 26, 79–85.
[143]
Liberski, P.P.; Yanagihara, R.; Gibbs, C.J., Jr.; Gajdusek, D.C. Re-evaluation of experimental Creutzfeldt-Jakob disease: Serial studies of the Fujisaki strain of Creutzfeldt-Jakob disease virus in mice. Brain 1990, 113, 121–137, doi:10.1093/brain/113.1.121.
[144]
Lampert, P.W.; Earle, K.M.; Gibbs, C.J., Jr.; Gajdusek, D.C. Experimental kuru encephalopathy in chimpanzees and spider monkey. J. Neuropathol. Exp. Neurol. 1969, 28, 353–370, doi:10.1097/00005072-196907000-00001.
[145]
Goodbrand, I.A.; Ironside, J.W.; Nicolson, D.; Bell, J.E. Prion protein accumulations in the spinal cords of patients with sporadic and growth hormone-associated Creutzfeldt-Jakob disease. Neurosci. Lett. 1995, 183, 127–130, doi:10.1016/0304-3940(94)11131-2.
[146]
Brandner, S.; Whitfield, J.; Boone, K.; Puva, A.; O’Malley, C.; Linehan, J.M.; Joiner, S.; Scaravilli, F.; Calder, I.; Alpers, M.P.; et al. Central and peripheral pathology of kuru: Pathological analysis of a recent case and comparison with other forms of human prion diseases. Phil. Trans. R. S. 2008, 363, 3755–3763, doi:10.1098/rstb.2008.0091.
[147]
Collinge, J.; Sidle, K.C.L.; Meads, J.; Ironside, J.W.; Hill, A.F. Molecular analysis of prion strain variation and the etiology of “new variant” CJD. Nature 1996, 383, 685–690, doi:10.1038/383685a0.
[148]
Parchi, P.; Castellani, R.; Capellari, S.; Ghetti, B.; Young, K.; Chen, S.G.; Farlow, M.; Dickson, D.W.; Sima, A.A.; Trojanowski, J.Q.; et al. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann. Neurol. 1996, 39, 767–778, doi:10.1002/ana.410390613.
[149]
Lee, H.-S.; Brown, P.; Cervenakova, L.; Garruto, R.; Alpers, M.P.; Gajdusek, D.C.; Goldafrb, L.G. Increased susceptibility to kuru of carriers of the PRNP 129 Methionine/Methionine genotype. J. Infect. Dis. 2001, 183, 192–196, doi:10.1086/317935.
[150]
Mead, S.; Poulter, M.; Uphill, J.; Beck, J.; Whitfield, J.; Webb, T.E.F.; Campbell, T.; Adamson, G.; Deriziotis, P.; Tabrizi, S.J.; et al. Genetic risk factors for variant Creutzfeldt-Jakob disease: A genome-wide association study. Lancet Neurol. 2009, 8, 57–66, doi:10.1016/S1474-4422(08)70265-5.
[151]
Mead, S.; Stumpf, M.P.; Whitfield, J.; Beck, J.A.; Poulter, M.; Campbell, T.; Uphill, J.B.; Goldstein, D.; Alpers, M.; Fisher, E.M.; et al. Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics. Science 2003, 300, 640–643, doi:10.1126/science.1083320.
[152]
Mead, S.; Whitfield, J.; Poulter, M.; Shah, P.; Uphill, J.; Beck, J.; Campbell, T.; al-Dujaly, H.; Hummerich, H.; Alpers, M.P.; et al. Genetic susceptibility, evolution and the kuru epdemic. Phil. Trans. R. S. 2008, 363, 3741–3746, doi:10.1098/rstb.2008.0087.
[153]
Mead, S. Prion disease genetics. Eur. J. Human Genet. 2006, 14, 1–9, doi:10.1038/sj.ejhg.5201544.
[154]
Lantos, B.; Bhata, K.; Doey, L.J.; al-Sarraj, S.; Doshi, R.; Beck, J.; Collinge, J. Is the neuropathology of new variant Creutzfeldt-Jakob disease and kuru similar? Lancet 1997, 350, 187–188.
[155]
Aguzzi, A.; Heikenwalder, M. Prion diseases: Cannibals and garbage piles. Nature 2003, 423, 127–129, doi:10.1038/423127a.
[156]
Brookfield, J.F. Human evolution: A legacy of cannibalism in our genes? Curr. Biol. 2003, 13, R592–R593, doi:10.1016/S0960-9822(03)00522-0.
[157]
Mead, S.; Whitfield, J.; Poulter, M.; Shah, P.; Uphill, J.; Campbell, T.; al-Dujaily, H.; Hummerich, H.; Beck, J.; Mein, C.A.; et al. A novel protective prion protein variant that colocalizes with kuru exposure. N. Engl. J. Med. 2009, 361, 2056–2065, doi:10.1056/NEJMoa0809716.
[158]
Mead, S.; Uphill, J.; Beck, J.; Poulter, M.; Campbell, T.; Lowe, J.; Adamson, G.; Hummerich, H.; Klopp, N.; Rückert, I.M.; et al. Genome-wide association study in multiple human prion diseases suggests genetic risk factors additional to PRNP. Hum. Mol. Genet. 2012, 21, 1897–1906, doi:10.1093/hmg/ddr607.
[159]
Marlar, R.A.; Leonard, B.L.; Billman, B.R.; Lambert, P.M.; Marlar, J.E. Biochemical evidence of cannibalism at a prehistoric Puebloan site in southwestern Colorado. Nature 2000, 407, 25–26, doi:10.1038/35024175.
[160]
Riel-Salvatore, J. Mad Neanderthal disease? Some comments on A potential role for Transmissible Spongiform Encephalopathies in Neanderthal extinction. Med. Hyp. 2008, 71, 473–474, doi:10.1016/j.mehy.2008.04.015.
[161]
Underdown, S. A potential role for transmissible spongiform encephalopathies in Neanderthal extinction. Med. Hyp. 2008, 71, 4–7, doi:10.1016/j.mehy.2007.12.014.
[162]
Wadsworth, J.D.F.; Joiner, S.; Linehan, J.M.; Asante, E.A.; Brandner, S.; Collinge, J. The origin of the prion agent of kuru: Molecular and biological strain typing. Phil. Trans. R. Soc. 2008, 363, 3747–3753, doi:10.1098/rstb.2008.0069.
[163]
Wadsworth, J.D.F.; Joiner, S.; Linehan, J.M.; Desbruslais, M.; Fox, K.; Cooper, S.; Cronier, S.; Asante, E.A.; Mead, S.; Brandner, S.; et al. Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice. Proc. Natl. Acad. Sci. USA 2007, 105, 3885–3890.
[164]
Hill, A.F.; Desbruslais, M.; Joiner, S.; Sidle, K.L.C.; Gowland, I.; Collinge, J.; Doey, L.J.; Lantos, P. The same prion strain causes vCJD and BSE. Nature 1997, 389, 448–450, doi:10.1038/38925.
[165]
Parchi, P.; Cescatti, M.; Notari, S.; Schulz-Schaeffer, W.J.; Capellari, S.; Giese, A.; Zou, W.Q.; Kretzschmar, H.; Ghetti, B.; Brown, P. Agent strain variation in human prion disease: Insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease. Brain 2010, 133, 3030–3042, doi:10.1093/brain/awq234.
[166]
Parchi, P; Saverioni, D. Molecular pathology, classification, and diagnosis of sporadic human prion disease variants. Folia Neuropathol 2012, 50, 20–45.
[167]
Manuelidis, L.; Chakrabarty, T.; Miyazawa, K.; Nduom, N.A.; Emmerling, K. The kuru infectious agent is a unique geographic isolate distinct from Creutzfeldt-Jakob disease and scrapie agents. Proc. Natl. Acad. Sci. USA 2009, 106, 13529–13534, doi:10.1073/pnas.0905825106.
