{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T17:52:21Z","timestamp":1761155541440},"publisher-location":"Berlin, Heidelberg","reference-count":101,"publisher":"Springer Berlin Heidelberg","isbn-type":[{"type":"print","value":"9783642765421"},{"type":"electronic","value":"9783642765407"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1991]]},"DOI":"10.1007\/978-3-642-76540-7_1","type":"book-chapter","created":{"date-parts":[[2011,11,19]],"date-time":"2011-11-19T21:56:04Z","timestamp":1321739764000},"page":"1-20","source":"Crossref","is-referenced-by-count":17,"title":["The Human Spongiform Encephalopathies: Kuru, Creutzfeldt-Jakob Disease, and the Gerstmann-Str\u00e4ussler-Scheinker Syndrome"],"prefix":"10.1007","author":[{"given":"P.","family":"Brown","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D. C.","family":"Gajdusek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","reference":[{"key":"1_CR1","first-page":"234","volume-title":"The central nervous system","author":"MP Alpers","year":"1968","unstructured":"Alpers MP (1968) Kuru: implications of its transmissibility for the interpretation of its changing epidemiologic pattern. In: The central nervous system. International Academy of Pathology Monograph No. 9, Baltimore, pp 234\u2013251"},{"key":"1_CR2","doi-asserted-by":"crossref","first-page":"852","DOI":"10.4269\/ajtmh.1965.14.852","volume":"14","author":"MP Alpers","year":"1965","unstructured":"Alpers MP, Gajdusek DC (1965) Changing patterns of kuru: epidemiological changes in the periods of increasing contact of the Fore people with Western civilization. Am J Trop Med Hyg 14: 852\u2013879","journal-title":"Am J Trop Med Hyg"},{"key":"1_CR3","volume-title":"Bibliography of kuru","author":"MP Alpers","year":"1975","unstructured":"Alpers MP, Gajdusek DC, Ono SG (1975) Bibliography of kuru, 3rd edn. National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda","edition":"3"},{"key":"1_CR4","first-page":"73","volume":"5","author":"S Araki","year":"1989","unstructured":"Araki S (1989) Familial amyloidotic polyneuropathy, Japanese type. Disc Neurosci 5: 73\u201379","journal-title":"Disc Neurosci"},{"key":"1_CR5","doi-asserted-by":"publisher","first-page":"1056","DOI":"10.1016\/S0140-6736(66)92031-9","volume":"2","author":"E Beck","year":"1966","unstructured":"Beck E, Daniel PM, Alpers M, Gajdusek DC, Gibbs CJ Jr (1966a) Experimental \u201ckuru\u201d in chimpanzees: a pathological report. Lancet 2:1056\u20131059","journal-title":"Lancet"},{"key":"1_CR6","series-title":"International congress series","volume-title":"Proceedings of the fifth international congress of neuropathology","author":"E Beck","year":"1966","unstructured":"Beck E, Daniel PM, Gajdusek DC (1966b) A comparison between the neuropathological changes in kuru and scrapie, a system degeneration. In: Proceedings of the fifth international congress of neuropathology. Excerpta Medica, Amsterdam (International congress series, no 100)"},{"key":"1_CR7","doi-asserted-by":"publisher","first-page":"441","DOI":"10.1093\/brain\/96.3.441","volume":"96","author":"E Beck","year":"1973","unstructured":"Beck E, Daniel PM, Gajdusek DC, Gibbs CJ Jr (1973) Experimental kuru in the chimpanzee: an neuropathological study. Brain 96: 441\u2013442","journal-title":"Brain"},{"key":"1_CR8","doi-asserted-by":"publisher","first-page":"585","DOI":"10.1093\/brain\/98.4.595","volume":"98","author":"E Beck","year":"1975","unstructured":"Beck E, Bak IJ, Christ JF, Gajdusek DC, Gibbs CJ Jr, Hassler R (1975) Experimental kuru in the spider monkey. Histopathological and ultrastructural studies of the brain during early stages of incubation. Brain 98: 585\u2013612","journal-title":"Brain"},{"key":"1_CR9","doi-asserted-by":"publisher","first-page":"755","DOI":"10.1093\/brain\/105.4.755","volume":"104","author":"E Beck","year":"1982","unstructured":"Beck E, Daniel PM, Davey A, Gajdusek DC, Gibbs CJ Jr (1982) The pathogenesis of spongiform encephalopathies: an ultrastructural study. Brain 104: 755\u2013786","journal-title":"Brain"},{"key":"1_CR10","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1093\/oxfordjournals.epirev.a036218","volume":"2","author":"P Brown","year":"1980","unstructured":"Brown P (1980) An epidemiologic critique of Creutzfeldt-Jakob disease. Epidemiol Rev 2: 113\u2013135","journal-title":"Epidemiol Rev"},{"key":"1_CR11","doi-asserted-by":"crossref","first-page":"895","DOI":"10.1212\/WNL.37.6.895","volume":"37","author":"P Brown","year":"1987","unstructured":"Brown P, Cathala F, Raubertas R, Gajdusek DC, Castaigne P (1987) The epidemiology of Creutzfeldt-Jakob disease: conclusion of a 15-year investigation in France and review of the world literature. Neurology 37: 895\u2013904","journal-title":"Neurology"},{"key":"1_CR12","volume-title":"Novel infections agents and the central nervous system","author":"P Brown","year":"1988","unstructured":"Brown P (1988) The clinical neurology and epidemiology of Creutzfeldt-Jakob disease, with special reference to iatrogenic cases. In: Bock G, Marsh J (eds) Novel infections agents and the central nervous system. Wiley, Chichester"},{"key":"1_CR13","doi-asserted-by":"publisher","first-page":"633","DOI":"10.1111\/j.1445-5994.1990.tb00390.x","volume":"20","author":"P Brown","year":"1990","unstructured":"Brown P (1990) Iatrogenic Creutzfeldt-Jakob disease. Aust NZ J Med 20: 633\u2013635","journal-title":"Aust NZ J Med"},{"key":"1_CR14","doi-asserted-by":"publisher","first-page":"295","DOI":"10.1002\/ana.410160305","volume":"16","author":"P Brown","year":"1984","unstructured":"Brown P, Rodgers-Johnson P, Cathala F, Gibbs CJ Jr, Gajdusek DC (1984) Creutzfeldt-Jakob disease of long duration: clinico-pathological charactieristics, transmissibility, and differential diagnosis. Ann Neurol 16: 295\u2013304","journal-title":"Ann Neurol"},{"key":"1_CR15","doi-asserted-by":"publisher","first-page":"597","DOI":"10.1002\/ana.410200507","volume":"20","author":"P Brown","year":"1986","unstructured":"Brown P, Cathala F, Castaigne P, Gajdusek DC (1986) Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. Ann Neurol 20: 597\u2013602","journal-title":"Ann Neurol"},{"key":"1_CR16","doi-asserted-by":"publisher","first-page":"467","DOI":"10.1093\/infdis\/161.3.467","volume":"161","author":"P Brown","year":"1990","unstructured":"Brown P, Wolff A, Liberski PP, Gajdusek DC (1990) Resistance of scrapie infectivity to steam autoclaving after formaldehyde fixation, and limited survival after ashing at 360\u00b0C: practical and theoretical implications. J Infect Dis 161: 467\u2013472","journal-title":"J Infect Dis"},{"key":"1_CR17","doi-asserted-by":"publisher","first-page":"1019","DOI":"10.1016\/0140-6736(91)92670-W","volume":"337","author":"P Brown","year":"1991","unstructured":"Brown P, Goldfarb LG, Gajdusek DC (1991) The neurobiology of spongiform encephalopathy: infectious amyloidoses with a genetic twist. Lancet 337:1019\u20131022","journal-title":"Lancet"},{"key":"1_CR18","doi-asserted-by":"publisher","first-page":"354","DOI":"10.1016\/S0140-6736(86)92319-6","volume":"1","author":"JM Candy","year":"1986","unstructured":"Candy JM, Oakley AE, Klinowski J, Carpenter TA, Perry RH, Atack JR, Perry K, Blessed G, Fairbairn A, Edwardson JA (1986) Aluminosilicates and senile plaque formation in Alzheimer\u2019s disease. Lancet 1:354\u2013357","journal-title":"Lancet"},{"volume-title":"Unconventional virus diseases of the central nervous system","year":"1989","key":"1_CR19","unstructured":"Court LA, Dormont D, Brown P, Kingsbury D (eds) (1989) Unconventional virus diseases of the central nervous system. Commissariat \u00e0 l\u2019Energie Atomique (CEA), Service de Documentation, Fontenay-aux-Roses, France"},{"key":"1_CR20","first-page":"95","volume":"3","author":"H Diringer","year":"1986","unstructured":"Diringer H, Braig HR, Pocchiari M, Bode L (1986) Scrapie-associated fibrils in the pathogenesis of diseases caused by unconventional slow viruses. Disc Neurosci 3: 95\u2013100","journal-title":"Disc Neurosci"},{"key":"1_CR21","doi-asserted-by":"publisher","first-page":"974","DOI":"10.1016\/0006-291X(89)92317-6","volume":"2","author":"K Dohura","year":"1989","unstructured":"Dohura K, Tateishi J, Sasaki H, Kitamoto T, Sakaki Y (1989) Proleu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Str\u00e4ussler syndrome. Biochem Biophys Res Commun 2: 974\u2013979","journal-title":"Biochem Biophys Res Commun"},{"key":"1_CR22","doi-asserted-by":"crossref","first-page":"1446","DOI":"10.1212\/WNL.39.11.1446","volume":"39","author":"MR Farlow","year":"1989","unstructured":"Farlow MR, Yee RD, Delouhy SR, Conneally PM, Azzarelli B, Ghetti B (1989) Gerstmann-Str\u00e4ussler-Scheinker disease. I. Extending the clinical spectrum. Neurology 39:1446\u20131452","journal-title":"Neurology"},{"volume-title":"Kuru: early letters and field notes from the collection of D. Carleton Gajdusek","year":"1980","key":"1_CR23","unstructured":"Farquhar J, Gajdusek DC (eds) (1980) Kuru: early letters and field notes from the collection of D. Carleton Gajdusek. Raven, New York"},{"key":"1_CR24","first-page":"79","volume":"5","author":"B Frangione","year":"1989","unstructured":"Frangione B (1989) Systemic amyloidosis and cerebral amyloid angiopathy. Disc Neurosci 5: 79\u201384","journal-title":"Disc Neurosci"},{"key":"1_CR25","doi-asserted-by":"publisher","first-page":"151","DOI":"10.1016\/0035-9203(63)90057-9","volume":"57","author":"DC Gajdusek","year":"1963","unstructured":"Gajdusek DC (1963) Kuru. Trans R Soc Trop Med Hyg 57:151\u2013169","journal-title":"Trans R Soc Trop Med Hyg"},{"key":"1_CR26","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1136\/jcp.25.Suppl_6.78","volume":"25","author":"DC Gajdusek","year":"1972","unstructured":"Gajdusek DC (1972) Spongiform virus encephalopathies. J Clin Pathol (Suppl) 25: 78\u201383","journal-title":"J Clin Pathol (Suppl)"},{"key":"1_CR27","volume-title":"Tropical neurology","author":"DC Gajdusek","year":"1973","unstructured":"Gajdusek DC (1973) Kuru in the New Guinea highlands. In: Spillane JD (ed) Tropical neurology. Oxford, New York"},{"volume-title":"Correspondence on the discovery and original investigations of kuru. Smadel-Gajdusek correspondence 1956\u20131959","year":"1976","key":"1_CR28","unstructured":"Gajdusek DC (ed) (1976) Correspondence on the discovery and original investigations of kuru. Smadel-Gajdusek correspondence 1956\u20131959. National Institutes of Health, Bethesda"},{"key":"1_CR29","doi-asserted-by":"publisher","first-page":"943","DOI":"10.1126\/science.142303","volume":"197","author":"DC Gajdusek","year":"1977","unstructured":"Gajdusek DC (1977) Unconventional viruses and the origin and disappearance of kuru. Revised version. Science 197: 943\u2013960","journal-title":"Science"},{"key":"1_CR30","first-page":"283","volume":"72","author":"DC Gajdusek","year":"1978","unstructured":"Gajdusek DC (1978) Slow infections with unconventional viruses. Harvey Lect 72: 283\u2013353","journal-title":"Harvey Lect"},{"key":"1_CR31","volume-title":"Subviral pathogens of plants and animals: viroids and prions","author":"DC Gajdusek","year":"1985","unstructured":"Gajdusek DC (1985) Subacute spongiform virus encephalopathies caused by unconventional viruses. In: Maramorosch K (ed) Subviral pathogens of plants and animals: viroids and prions. Academic, New York"},{"key":"1_CR32","volume-title":"Nutrition, health and peace","author":"DC Gajdusek","year":"1987","unstructured":"Gajdusek DC (1987) A newly recognized mechanism of pathogenesis in Alzheimer\u2019s disease, amyotrophic lateral sclerosis, and other degenerative neurological diseases: the \u00df-fibrilloses of brain. In: Jariwalla RJ, Schwoebel SL (eds) Nutrition, health and peace. Linus Pauling Institute, Palo Alto"},{"key":"1_CR33","volume-title":"Genetics and Alzheimer\u2019s disease","author":"DC Gajdusek","year":"1988","unstructured":"Gajdusek DC (1988a) Etiology versus pathogenesis: the cause of post-translational modifications of host specified brain proteins to amyloid configuration. In: Sinet PM, Lamour Y, Christen Y (eds) Genetics and Alzheimer\u2019s disease. Proceedings of a meeting held by the foundation IPSEN pour la Recherche Th\u00e9rapeutique, Paris. Springer, Berlin Heidelberg New York"},{"key":"1_CR34","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1016\/0165-5728(88)90140-3","volume":"20","author":"DC Gajdusek","year":"1988","unstructured":"Gajdusek DC (1988b) Transmissible and non-transmissible amyloidoses: autocatalytic post-translational conversion of host precursor proteins to \u00df-pleated configurations. J Neuroimmunol 20:95\u2013110","journal-title":"J Neuroimmunol"},{"key":"1_CR35","volume-title":"Journals 1957\u20131989, 39 volumes","author":"DC Gajdusek","year":"1957","unstructured":"Gajdusek DC (1957\u20131989) Journals 1957\u20131989, 39 volumes. National Institutes of Health, Bethesda"},{"key":"1_CR36","volume-title":"Modern trends in human leukemia VIII","author":"DC Gajdusek","year":"1989","unstructured":"Gajdusek DC (1989) Fantasy of a \u201cvirus\u201d from the inorganic world: pathogenesis of cerebral amyloidoses by polymer nucleating agents and\/or \u201cviruses\u201d. In: Neth R, Gallo RC, Greaves MF, Gacdicke G, Ritter J (eds) Modern trends in human leukemia VIII. Springer, Berlin Heidelberg New York"},{"key":"1_CR37","volume-title":"Virology","author":"DC Gajdusek","year":"1990","unstructured":"Gajdusek DC (1990) Subacute spongiform encephalopathies: transmissible cerebral amyloidoses caused by unconventional viruses. In: Virology, 2nd edn. Raven, New York","edition":"2"},{"key":"1_CR38","volume-title":"Proceedings of European Commission Seminar on Sub-Acute spongiform encephalopathies, Brussels, November 13, 1990","author":"DC Gajdusek","year":"1991","unstructured":"Gajdusek DC (1991) Transthyretin amyloidoses of familial amyloidotic polyneuropathy as a paradigm for the genetic control of de novo generation of Creutzfeldt-Jakob disease infectious amyloid by a spontaneous change in configuration of host precursor protein. In: Marchant BA (ed) Proceedings of European Commission Seminar on Sub-Acute spongiform encephalopathies, Brussels, November 13, 1990"},{"key":"1_CR39","doi-asserted-by":"publisher","first-page":"588","DOI":"10.1038\/230588a0","volume":"230","author":"DC Gajdusek","year":"1971","unstructured":"Gajdusek DC, Gibbs CJ Jr (1971) Transmission of the two subacute spongiform encephalopathies of man (kuru and Creutzfeldt-Jakob disease) to New World monkeys. Nature 230: 588\u2013591","journal-title":"Nature"},{"key":"1_CR40","first-page":"279","volume":"8","author":"DC Gajdusek","year":"1973","unstructured":"Gajdusek DC, Gibbs CJ Jr (1973) Subacute and chronic diseases caused by atypical infections with unconventional viruses in aberrant hosts. Perspect Virol 8: 279\u2013311","journal-title":"Perspect Virol"},{"key":"1_CR41","volume-title":"The nervous system, vol 2: The clinical neurosciences","author":"DC Gajdusek","year":"1975","unstructured":"Gajdusek DC, Gibbs CJ Jr (1975) Slow virus infections of the nervous system and the laboratories of slow, latent and temperate virus infections. In: Chase TN (ed) The nervous system, vol 2: The clinical neurosciences. Raven, New York"},{"key":"1_CR42","doi-asserted-by":"publisher","first-page":"974","DOI":"10.1056\/NEJM195711142572005","volume":"257","author":"DC Gajdusek","year":"1957","unstructured":"Gajdusek DC, Zigas V (1957) Degenerative disease of the central nervous system in New Guinea: the endemic occurrence of \u201ckuru\u201d in the native population. N Engl J Med 257: 974\u2013978","journal-title":"N Engl J Med"},{"key":"1_CR43","doi-asserted-by":"publisher","first-page":"442","DOI":"10.1016\/0002-9343(59)90251-7","volume":"26","author":"DC Gajdusek","year":"1959","unstructured":"Gajdusek DC, Zigas V (1959) Kuru: clinical, pathological and epidemiological study of an acute progressive degenerative disease of the central nervous system among natives of the Eastern Highlands of New Guinea. Am J Med 26: 442\u2013469","journal-title":"Am J Med"},{"volume-title":"Slow, latent and temperate virus infections. NINDS monograph no 2, National Institutes of Health, PHS Publication no 1378, US Government Printing Office","year":"1965","key":"1_CR44","unstructured":"Gajdusek DC, Gibbs CJ Jr, Alpers MP (eds) (1965) Slow, latent and temperate virus infections. NINDS monograph no 2, National Institutes of Health, PHS Publication no 1378, US Government Printing Office, Washington, DC"},{"key":"1_CR45","doi-asserted-by":"publisher","first-page":"794","DOI":"10.1038\/209794a0","volume":"209","author":"DC Gajdusek","year":"1966","unstructured":"Gajdusek DC, Gibbs CJ Jr, Alpers MP (1966) Experimental transmission of a kuru-like syndrome in chimpanzees. Nature 209: 794\u2013796","journal-title":"Nature"},{"key":"1_CR46","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1126\/science.155.3759.212","volume":"155","author":"DC Gajdusek","year":"1967","unstructured":"Gajdusek DC, Gibbs CJ Jr, Alpers M (1967) Transmission and passage of experimental \u2018kuru\u2019 to chimpanzees. Science 155: 212\u2013214","journal-title":"Science"},{"key":"1_CR47","first-page":"711","volume":"315","author":"RM Garruto","year":"1985","unstructured":"Garruto RM, Swyt C, Yanagihara R, Fiori CE, Gajdusek DC (1985) Intraneural co-localization of silicon with calcium and aluminium in amyotrophic lateral sclerosis and Parkinsonism with dementia of Guam. New Engl J Med 315: 711\u2013712","journal-title":"New Engl J Med"},{"key":"1_CR48","doi-asserted-by":"publisher","first-page":"388","DOI":"10.1126\/science.161.3839.388","volume":"161","author":"CJ Gibbs Jr","year":"1968","unstructured":"Gibbs CJ Jr, Gajdusek DC, Asher DM, Alpers MP, Beck E, Daniel PM, Matthews WB (1968) Creutzfeldt-Jakob disease (subacute spongiform encephalopathy): transmission to the chimpanzee. Science 161: 388\u2013389","journal-title":"Science"},{"key":"1_CR49","doi-asserted-by":"publisher","first-page":"388","DOI":"10.1126\/science.161.3839.388","volume":"161","author":"CJ Gibbs Jr","year":"1979","unstructured":"Gibbs CJ Jr, Gajdusek DC, Amyx HL (1979) Strain variation in the viruses of Creutzfeldt-Jakob disease (subacute spongiform encephalopathy): transmission to the chimpanzee. Science 161:388\u2013389","journal-title":"Science"},{"key":"1_CR50","doi-asserted-by":"publisher","first-page":"205","DOI":"10.1093\/infdis\/142.2.205","volume":"142","author":"CJ Gibbs Jr","year":"1980","unstructured":"Gibbs CJ Jr, Amyx HL, Bacote A, Masters C, Gajdusek DC (1980) Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates. J Infect Dis 142: 205\u2013208","journal-title":"J Infect Dis"},{"key":"1_CR51","volume-title":"Cannibalism in the kuru region","author":"RM Glass","year":"1963","unstructured":"Glass RM (1963) Cannibalism in the kuru region. Department of Public Health, Papua New Guinea"},{"key":"1_CR52","doi-asserted-by":"publisher","first-page":"174","DOI":"10.1016\/0140-6736(90)91693-5","volume":"336","author":"LG Goldfarb","year":"1990","unstructured":"Goldfarb LG, Brown P, Goldgaber D, Garruto RM, Yanagihara R, Asher DM, Gajdusek DC (1990a) Identical mutation in unrelated patients with Creutzfeldt-Jakob disease. Lancet 336:174\u2013175","journal-title":"Lancet"},{"key":"1_CR53","doi-asserted-by":"publisher","first-page":"514","DOI":"10.1016\/0140-6736(90)92073-Q","volume":"336","author":"LG Goldfarb","year":"1990","unstructured":"Goldfarb LG, Mitrov\u00e1 E, Brown P, Toh BH, Gajdusek DC (1990b) Mutation in condon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in Slovakia. Lancet 336:514\u2013515","journal-title":"Lancet"},{"key":"1_CR54","doi-asserted-by":"publisher","first-page":"637","DOI":"10.1016\/0140-6736(90)93443-S","volume":"336","author":"LG Goldfarb","year":"1990","unstructured":"Goldfarb LG, Korczyn AD, Brown P, Chapman J, Gajdusek DC (1990c) Mutation in codon 200 of scrapie amyloid precursor gene linked to Creutzfeldt-Jakob disease in Sephardic Jews of Libyan and non-Libyan origin. Lancet 336: 637\u2013638","journal-title":"Lancet"},{"key":"1_CR55","first-page":"247","volume":"108","author":"LG Goldfarb","year":"1990","unstructured":"Goldfarb LG, Brown P, Goldgaber D, Asher DM, Rubenstein R, Brown WT, Piccardo P, Kascsak, Boellaard, Gajdusek DC (1990d) Creutzfeldt-Jakob disease and kuru patients lack a mutation consistently found in the Gerstmann-Str\u00e4ussler-Scheinker syndrome. Exp. Neurol 108: 247\u2013250","journal-title":"Neurol"},{"key":"1_CR56","doi-asserted-by":"publisher","first-page":"425","DOI":"10.1016\/0140-6736(91)91198-4","volume":"337","author":"LG Goldfarb","year":"1991","unstructured":"Goldfarb LG, Haltia M, Brown P, Nieto A, Kovanen J, McCombie WR, Trapp S, Gajdusek DC (1991) New mutation in scrapie amyloid precursor gene in Finnish Creutzfeldt-Jakob disease kindred. Lancet 337: 425","journal-title":"Lancet"},{"key":"1_CR57","doi-asserted-by":"publisher","first-page":"877","DOI":"10.1126\/science.3810169","volume":"235","author":"D Goldgaber","year":"1987","unstructured":"Goldgaber D, Lerman Ml, McBride OW, Saffiotti U, Gajdusek DC (1987) Characterization and chromosomal localization of cDNA encoding brain amyloid of Alzheimer\u2019s disease. Science 235: 877\u2013880","journal-title":"Science"},{"key":"1_CR58","doi-asserted-by":"publisher","first-page":"204","DOI":"10.1016\/0014-4886(89)90095-2","volume":"106","author":"D Goldgaber","year":"1989","unstructured":"Goldgaber D, Goldfarb LG, Brown P, Asher DM, Brown WT, Lin S, Teener JW, Feinstone SM, Rubenstein R, Kascsak RJ, Boellaard JW, Gajdusek DC (1989) Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Str\u00e4ussler-Scheinker\u2019s syndrome. Exp Neurol 106:204\u2013206","journal-title":"Exp Neurol"},{"key":"1_CR59","first-page":"69","volume":"5","author":"DC Guiroy","year":"1989","unstructured":"Guiroy DC, Gajdusek DC (1989) Fibril-derived amyloid enhancing factors as nucleating agents in Alzheimer\u2019s disease and transmissible virus dementia. Disc Neurosci 5: 69\u201373","journal-title":"Disc Neurosci"},{"key":"1_CR60","doi-asserted-by":"publisher","first-page":"289","DOI":"10.1016\/S0140-6736(59)92081-1","volume":"2","author":"WJ Hadlow","year":"1959","unstructured":"Hadlow WJ (1959) Scrapie and kuru. Lancet 2: 289\u2013290","journal-title":"Lancet"},{"key":"1_CR61","doi-asserted-by":"publisher","first-page":"381","DOI":"10.1016\/0022-510X(79)90171-0","volume":"42","author":"M Haltia","year":"1979","unstructured":"Haltia M, Kovanen J, van Crevel H, Bots GThAM, Stefanko S. Familial Creutzfeldt-Jakob disease (1979) J Neurol Sci 42: 381\u2013389","journal-title":"J Neurol Sci"},{"key":"1_CR62","doi-asserted-by":"publisher","first-page":"387","DOI":"10.1093\/infdis\/115.4.387","volume":"115","author":"GR Hartsough","year":"1965","unstructured":"Hartsough GR, Burger D (1965) Encephalopathy of mink. I. Epizootiologic and clinical observations. J Infect Dis 115:387\u2013392","journal-title":"J Infect Dis"},{"key":"1_CR63","first-page":"226","volume":"18","author":"RW Hornabrook","year":"1975","unstructured":"Hornabrook RW, Wagner F (1975) Creutzfeldt-Jakob disease. PNG Med J 18: 226\u2013228","journal-title":"PNG Med J"},{"key":"1_CR64","doi-asserted-by":"publisher","first-page":"1587","DOI":"10.1126\/science.1980379","volume":"250","author":"KK Hsiao","year":"1990","unstructured":"Hsiao KK, Scott M, Foster D, Groth DF, DeArmond SJ, Prusiner SB (1990) Spontaneous neurodegeneration in transgenic mice with mutant prion protein. Science 250: 1587\u20131590","journal-title":"Science"},{"key":"1_CR65","doi-asserted-by":"publisher","first-page":"342","DOI":"10.1038\/338342a0","volume":"338","author":"K Hsiao","year":"1989","unstructured":"Hsiao K, Baker HF, Crow TJ, Poulter M, Owen F, Terwilliger JD, Westaway D, Ott J, Prusiner SB (1989) Linkage of a prion protein missense varient to Gerstmann-Str\u00e4ussler syndrome. Nature 338: 342\u2013345","journal-title":"Nature"},{"key":"1_CR66","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1212\/WNL.41.5.681","volume":"41","author":"KK Hsiao","year":"1991","unstructured":"Hsiao KK, Cass C, Schallenberg GD, Bird T, Devine-Gage E, Wisniewski H, Prusiner SB (1991) A prion protein variant in a family with telecephalic form of Gerstmann-Str\u00e4ussler-Scheinker syndrome. Neurology 41: 681\u2013684","journal-title":"Neurology"},{"key":"1_CR67","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1126\/science.183.4120.90","volume":"183","author":"E Kahana","year":"1974","unstructured":"Kahana E, Alter M, Braham J, Sofer D (1974) Creutzfeldt-Jakob disease: focus among Libyan Jews in Israel. Science 183: 90\u201391","journal-title":"Science"},{"key":"1_CR68","first-page":"30","volume":"54","author":"RH Kimberlin","year":"1990","unstructured":"Kimberlin RH (1990) Transmissible encephalopathies in animals. Can J Vet Res 54: 30\u201337","journal-title":"Can J Vet Res"},{"key":"1_CR69","first-page":"799","volume":"8","author":"I Klatzo","year":"1959","unstructured":"Klatzo I, Gajdusek DC, Zigas V (1959) Pathology of kuru. Lab Invest 8: 799\u2013847","journal-title":"Lab Invest"},{"key":"1_CR70","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1159\/000110837","volume":"3","author":"RL Klitzman","year":"1984","unstructured":"Klitzman RL, Alpers MP, Gajdusek DC (1984) The natural incubation period of kuru and the episodes of transmission in three clusters of patients. Neuroepidemiology 3: 13\u201320","journal-title":"Neuroepidemiology"},{"key":"1_CR71","first-page":"626","volume":"68","author":"PW Lampert","year":"1972","unstructured":"Lampert PW, Gajdusek DC, Gibbs CJ Jr (1972) Subacutre spongiform virus encephalopathies: scrapie, kuru and Creutzfeldt-Jakob disease. Am J Pathol 68: 626\u2013646","journal-title":"Am J Pathol"},{"key":"1_CR72","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1212\/WNL.31.5.538","volume":"31","author":"DMD Landis","year":"1981","unstructured":"Landis DMD, Williams RS, Masters CL (1981) Golgi and electron microscopic studies of spongiform encephalopathy. Neurology 31: 538\u2013549","journal-title":"Neurology"},{"key":"1_CR73","volume-title":"Kuru sorcery","author":"S Lindenbaum","year":"1979","unstructured":"Lindenbaum S (1979) Kuru sorcery. Mayfield, Palo Alto"},{"key":"1_CR74","doi-asserted-by":"publisher","first-page":"177","DOI":"10.1002\/ana.410050212","volume":"5","author":"CL Masters","year":"1979","unstructured":"Masters CL, Harris JO, Gajdusek DC, Gibbs CJ Jr, Bernoulli C, Asher DM (1979) Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol 5: 177\u2013188","journal-title":"Ann Neurol"},{"key":"1_CR75","doi-asserted-by":"publisher","first-page":"559","DOI":"10.1093\/brain\/104.3.559","volume":"104","author":"CL Masters","year":"1981","unstructured":"Masters CL, Gajdusek DC, Gibbs CJ Jr (1981) Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Str\u00e4ussler syndrome, with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathies. Brain 104: 559\u2013588","journal-title":"Brain"},{"key":"1_CR76","volume-title":"Viral diseases of the central nervous system","author":"WB Matthews","year":"1975","unstructured":"Matthews WB (1975) The clinical aspects of slow virus infections of the human brain. In: Jllis LS (ed) Viral diseases of the central nervous system. Bailliere Tindall, London"},{"key":"1_CR77","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1126\/science.239.4838.385","volume":"239","author":"A McPherson","year":"1988","unstructured":"McPherson A, Shlichta P (1988) Heterogeneous and epitaxial nucleation of protein crystals on mineral surfaces. Science 239: 385\u2013387","journal-title":"Science"},{"key":"1_CR78","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1007\/BF00691333","volume":"54","author":"PA Merz","year":"1981","unstructured":"Merz PA, Somerville RA, Wisniewski HM, Iqbal K (1981) Abnormal fibrils from scrapie infected brain. Acta Neuropathol (Berl) 54: 63\u201374","journal-title":"Acta Neuropathol (Berl)"},{"key":"1_CR79","doi-asserted-by":"publisher","first-page":"327","DOI":"10.1097\/00005072-198305000-00075","volume":"42","author":"PA Merz","year":"1983","unstructured":"Merz PA, Rohwer RG, Somerville RA, Wisniewski HM, Gibbs CJ Jr, Gajdusek DC (1983a) Scrapie associated fibrils in human Creutzfeldt-Jakob disease. J Neuropathol Exp Neurol 42: 327","journal-title":"J Neuropathol Exp Neurol"},{"key":"1_CR80","volume-title":"Virus non conventionnels et affections du syst\u00e8me nerveux central","author":"PA Merz","year":"1983","unstructured":"Merz PA, Somerville RA, Wisniewski HM (1983b) Abnormal fibrils in scrapie and senile dementia of Alzheimer\u2019s type. In: Court L, Cathala F (eds) Virus non conventionnels et affections du syst\u00e8me nerveux central. Masson, Paris"},{"key":"1_CR81","doi-asserted-by":"publisher","first-page":"474","DOI":"10.1038\/306474a0","volume":"306","author":"PA Merz","year":"1983","unstructured":"Merz PA, Somerville RA, Wisniewski HM, Manuelidis L, Manuelidis EE (1983c) Scrapie associated fibrils in Creutzfeldt-Jakob disease. Nature 306: 474\u2013476","journal-title":"Nature"},{"key":"1_CR82","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1126\/science.6377496","volume":"225","author":"PA Merz","year":"1984","unstructured":"Merz PA, Rohwer RG, Kascsak R, et al. (1984) An infection specific particle from the unconventional slow virus diseases. Science 225: 385\u2013387","journal-title":"Science"},{"key":"1_CR83","volume-title":"Unconventional virus diseases of the central nervous system. Commissariat \u00e0 l\u2019Energie Atomique (CEA), Service de Documentation","author":"E Mitrova","year":"1989","unstructured":"Mitrova E (1989) Analytical epidemiology and risk factors in CJD. In: Court LA, Dormont D, Brown P, Kingsbury DT (eds) Unconventional virus diseases of the central nervous system. Commissariat \u00e0 l\u2019Energie Atomique (CEA), Service de Documentation, Fontenay-aux-Roses, France"},{"key":"1_CR84","doi-asserted-by":"publisher","first-page":"7730","DOI":"10.1073\/pnas.84.21.7730","volume":"84","author":"HK Narang","year":"1987","unstructured":"Narang HK, Asher DM, Gajdusek DC (1987) Tubulofilaments in negatively stained scrapie-infected brains: relationship to scrapie associated fibrils. Proc Natl Acad Sci USA 84: 7730\u20137734","journal-title":"Proc Natl Acad Sci USA"},{"key":"1_CR85","doi-asserted-by":"publisher","first-page":"3575","DOI":"10.1073\/pnas.85.10.3575","volume":"85","author":"HK Narang","year":"1988","unstructured":"Narang HK, Asher DM, Gajdusek DC (1988) Evidence that DNA is present in abnormal tubulofilamentous structures found in scrapie. Proc Natl Acad Sci USA 85: 3575\u20133579","journal-title":"Proc Natl Acad Sci USA"},{"key":"1_CR86","doi-asserted-by":"publisher","first-page":"622","DOI":"10.1016\/0140-6736(91)91696-R","volume":"337","author":"A Nieto","year":"1991","unstructured":"Nieto A, Goldfarb LG, Brown P, McCombie WR, Trapp S, Asher DM, Gajdusek DC (1991) Codon 178 mutation in ethnically diverse Creutzfeldt-Jakob disease families. Lancet 337: 622\u2013623","journal-title":"Lancet"},{"key":"1_CR87","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1016\/0169-328X(90)90038-F","volume":"7","author":"F Owen","year":"1990","unstructured":"Owen F, Poulter M, Shah T, Collinge J, Lofthouse R, Baker H, Ridley R, McVey J, Crow TJ (1990) An in-frame insertion in the prion protein gene in familial Creutzfeldt-Jakob disease. Mol Brain Res 7: 273\u2013276","journal-title":"Mol Brain Res"},{"key":"1_CR88","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1016\/S0140-6736(89)91713-3","volume":"1","author":"F Owen","year":"1989","unstructured":"Owen F, Poulter M, Lofthouse R, Collinge J, Crow TJ, Risby D, Baker HF, Ridley RM, Hsaio K, Prusiner SB (1989) Insertion in prion protein gene in familial Creutzfeldt-Jakob disease. Lancet 1:51\u201352","journal-title":"Lancet"},{"key":"1_CR89","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1093\/oxfordjournals.aje.a115103","volume":"129","author":"RF Raubertas","year":"1989","unstructured":"Raubertas RF, Brown P, Cathala F, Brown I (1989) The question of clustering of Creutzfeldt-Jakob disease. Am J Epidemiol 129: 146\u2013154","journal-title":"Am J Epidemiol"},{"key":"1_CR90","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1002\/ana.410140104","volume":"14","author":"AM Salazar","year":"1983","unstructured":"Salazar AM, Masters CL, Gajdusek DC, Gibbs CJ Jr (1983) Syndromes of amyotrophic lateral sclerosis and dementia: relation to transmissible Creutzfeldt-Jakob disease. Ann Neurol 14: 17\u201326","journal-title":"Ann Neurol"},{"key":"1_CR91","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1007\/BF00695613","volume":"64","author":"J Tateishi","year":"1984","unstructured":"Tateishi J, Sato Y, Nagara H, Boellaard JW (1984) Experimental transmission of human subacute spongiform encephalopathy in small rodents. Acta Neuropathol 64: 55\u201358","journal-title":"Acta Neuropathol"},{"key":"1_CR92","doi-asserted-by":"crossref","first-page":"1578","DOI":"10.1212\/WNL.40.10.1578","volume":"40","author":"J Tateishi","year":"1990","unstructured":"Tateishi J, Kitamoto T, Dohura K, Sakaki Y, Steinmetz G, Tranchant C, Warter JM, Heldt N (1990) Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann-Str\u00e4ussler-Scheinker syndrome. Neurology 40: 1578\u20131581","journal-title":"Neurology"},{"key":"1_CR93","doi-asserted-by":"publisher","first-page":"239","DOI":"10.1007\/BF00150425","volume":"6","author":"G Trabattoni","year":"1990","unstructured":"Trabattoni G, Lechi A, Bettoni L, Macchi G, Brown P (1990) Considerations on a group of 13 patients with Creutzfeldt-Jakob disease in the region of Parma (Italy). Eur J Epidemiol 6: 239\u2013243","journal-title":"Eur J Epidemiol"},{"key":"1_CR94","volume-title":"Aging and dementia","author":"R Traub","year":"1977","unstructured":"Traub R, Gajdusek DC, Gibbs CJ Jr (1977) Transmissible virus dementia: the relation of transmissible spongiform encephalopathy to Creutzfeldt-Jakob disease. In: Kinsbourne M, Smith L (eds) Aging and dementia. Spectrum, New York"},{"key":"1_CR95","first-page":"393","volume-title":"Amyloid and Amyloidoses","author":"S Wakasugi","year":"1988","unstructured":"Wakasugi S, Inomoto T, Yi S et al. (1988) Potential animal model for familial amyloidotic polyneuropathy through introduction of human mutant transthyretin gene into mice. In: Takashi I, Shakuro A, Fumike O, Shumuko K, Eiro T (eds) Amyloid and Amyloidoses. Plenum Press, New York, pp 393\u2013398"},{"key":"1_CR96","doi-asserted-by":"publisher","first-page":"419","DOI":"10.1136\/vr.121.18.419","volume":"121","author":"GA Wells","year":"1987","unstructured":"Wells GA, Scott AC, Johnson CT, Gunning RF, Hancock RD, Jeffrey M, Dawson M, Bradley R (1987) A novel progressive spongiform encephalopathy in cattle. Vet Rec 121:419\u2013420","journal-title":"Vet Rec"},{"key":"1_CR97","doi-asserted-by":"publisher","first-page":"134","DOI":"10.1136\/jnnp.47.2.134","volume":"47","author":"RG Will","year":"1979","unstructured":"Will RG, Matthews WB (1979) A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970\u20131979. J Neurol Neurosurg Psychiatry 47: 134\u2013140","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"1_CR98","first-page":"638","volume":"123","author":"JW Wilesmith","year":"1988","unstructured":"Wilesmith JW, Wells GAH, Cranwell MP, Ryan JBM (1988) Bovine spongiform encephalopathy: epidemiological studies. Vet Rec 123: 638\u2013644","journal-title":"Vet Rec"},{"key":"1_CR99","doi-asserted-by":"crossref","first-page":"745","DOI":"10.5694\/j.1326-5377.1957.tb60287.x","volume":"2","author":"V Zigas","year":"1957","unstructured":"Zigas V, Gajdusek DC (1957) Kuru: clinical study of a new syndrome resembling paralysis agitans in natives of the Eastern Highlands of Australian New Guinea. Med J Aust 2: 745\u2013754","journal-title":"Med J Aust"},{"key":"1_CR100","first-page":"58","volume-title":"Pathological similarity to human familial amyloidotic polyneuropathy (FAP) type I in transgenic mice carrying the human mutant transthyretin gene","author":"S Yi","year":"1990","unstructured":"Yi S, Takahashi K, Tashiro F, Wakasugi S, Yamamura K, Araki S (1990) Pathological similarity to human familial amyloidotic polyneuropathy (FAP) type I in transgenic mice carrying the human mutant transthyretin gene. Abstract no. 07\/5 in Programme and Abstracts of the VIth International Symposium on Amyloidosis, Oslo, Norway, August 5\u20138, p 58"},{"key":"1_CR101","volume-title":"Creutzfeldt-Jakob disease in Israel: an epidemiological evaluation","author":"N Zilber","year":"1991","unstructured":"Zilber N, Kahana E, Abraham M (1991) Creutzfeldt-Jakob disease in Israel: an epidemiological evaluation. Neurology (in press)"}],"container-title":["Current Topics in Microbiology and Immunology","Transmissible Spongiform Encephalopathies:"],"original-title":[],"link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/978-3-642-76540-7_1.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,17]],"date-time":"2021-12-17T06:22:18Z","timestamp":1639722138000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/978-3-642-76540-7_1"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1991]]},"ISBN":["9783642765421","9783642765407"],"references-count":101,"URL":"https:\/\/doi.org\/10.1007\/978-3-642-76540-7_1","relation":{},"ISSN":["0070-217X"],"issn-type":[{"type":"print","value":"0070-217X"}],"subject":[],"published":{"date-parts":[[1991]]}}}