{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T18:14:33Z","timestamp":1791137673369,"version":"4.1.0"},"reference-count":66,"publisher":"The Royal Society","issue":"1510","license":[{"start":{"date-parts":[[2008,11,27]],"date-time":"2008-11-27T00:00:00Z","timestamp":1227744000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/royalsociety.org\/journals\/ethics-policies\/data-sharing-mining\/"}],"content-domain":{"domain":["royalsocietypublishing.org"],"crossmark-restriction":true},"short-container-title":["Phil. Trans. R. Soc. B"],"published-print":{"date-parts":[[2008,11,27]]},"abstract":"<jats:p>Kuru is an acquired human prion disease that primarily affected the Fore linguistic group of the Eastern Highlands of Papua New Guinea. The central clinical feature of kuru is progressive cerebellar ataxia and, in sharp contrast to most cases of sporadic Creutzfeldt\u2013Jakob disease (CJD), dementia is a less prominent and usually late clinical feature. In this regard, kuru is more similar to variant CJD, which also has similar prodromal symptoms of sensory disturbance and joint pains in the legs and psychiatric and behavioural changes. Since a significant part of the clinicopathological diversity seen in human prion disease is likely to relate to the propagation of distinct human prion strains, we have compared the transmission properties of kuru prions with those isolated from patients with sporadic, iatrogenic and variant CJD in both transgenic and wild-type mice. These data have established that kuru prions have prion strain properties equivalent to those of classical (sporadic and iatrogenic) CJD prions but distinct from variant CJD prions. Here, we review these findings and discuss how peripheral routes of infection and other factors may be critical modifiers of the kuru phenotype.<\/jats:p>","DOI":"10.1098\/rstb.2008.0069","type":"journal-article","created":{"date-parts":[[2008,10,14]],"date-time":"2008-10-14T01:52:55Z","timestamp":1223949175000},"page":"3747-3753","update-policy":"https:\/\/doi.org\/10.1098\/crossmark-policy","source":"Crossref","is-referenced-by-count":42,"title":["The origin of the prion agent of kuru: molecular and biological strain typing"],"prefix":"10.1098","volume":"363","author":[{"given":"Jonathan D.F","family":"Wadsworth","sequence":"first","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan","family":"Joiner","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline M","family":"Linehan","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuel A","family":"Asante","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian","family":"Brandner","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Collinge","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"175","published-online":{"date-parts":[[2008,11,27]]},"reference":[{"key":"e_1_3_2_2_1","first-page":"451","volume-title":"Prions: novel infectious pathogens causing scrapie and Creutzfeldt\u2013Jakob disease","author":"Alpers M","year":"1987"},{"key":"e_1_3_2_3_1","first-page":"7","article-title":"Kuru and Creutzfeldt\u2013Jakob disease: clinical and aetiological aspects","volume":"8","author":"Alpers M","year":"1971","journal-title":"Proc. Aust. Assoc. Neurol"},{"key":"e_1_3_2_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/cdf653"},{"key":"e_1_3_2_5_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0604292103"},{"key":"e_1_3_2_6_1","doi-asserted-by":"crossref","first-page":"7859","DOI":"10.1128\/JVI.68.12.7859-7868.1994","article-title":"Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy","volume":"68","author":"Bessen R.A","year":"1994","journal-title":"J. Virol"},{"key":"e_1_3_2_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2990.1994.tb01169.x"},{"key":"e_1_3_2_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410160305"},{"key":"e_1_3_2_9_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.37.6.895"},{"key":"e_1_3_2_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(92)92431-E"},{"key":"e_1_3_2_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410350504"},{"key":"e_1_3_2_12_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.55.8.1075"},{"key":"e_1_3_2_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000231528.65069.3f"},{"key":"e_1_3_2_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/bmb\/66.1.99"},{"key":"e_1_3_2_15_1","doi-asserted-by":"publisher","DOI":"10.1038\/39057"},{"key":"e_1_3_2_16_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.22.13239"},{"key":"e_1_3_2_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/6.10.1699"},{"key":"e_1_3_2_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(99)05128-4"},{"key":"e_1_3_2_19_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.neuro.24.1.519"},{"key":"e_1_3_2_20_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2004.048660"},{"key":"e_1_3_2_21_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1138718"},{"key":"e_1_3_2_22_1","unstructured":"Collinge J& Palmer M.S Prion diseases. 1st edn. 1997 Oxford UK:Oxford University Press."},{"key":"e_1_3_2_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(91)93128-V"},{"key":"e_1_3_2_24_1","doi-asserted-by":"publisher","DOI":"10.1038\/378779a0"},{"key":"e_1_3_2_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/383685a0"},{"key":"e_1_3_2_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(06)68930-7"},{"key":"e_1_3_2_27_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awl159"},{"key":"e_1_3_2_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(04)17143-2"},{"key":"e_1_3_2_29_1","doi-asserted-by":"publisher","DOI":"10.1093\/bmb\/66.1.213"},{"key":"e_1_3_2_30_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1973.00490290058006"},{"key":"e_1_3_2_31_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1750-3639.1997.tb01072.x"},{"key":"e_1_3_2_32_1","doi-asserted-by":"publisher","DOI":"10.1159\/000060375"},{"key":"e_1_3_2_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tim.2003.10.007"},{"key":"e_1_3_2_34_1","doi-asserted-by":"publisher","DOI":"10.1038\/38925"},{"key":"e_1_3_2_35_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awg125"},{"key":"e_1_3_2_36_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awl013"},{"key":"e_1_3_2_37_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.1580"},{"key":"e_1_3_2_38_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-002-0896-9"},{"key":"e_1_3_2_39_1","doi-asserted-by":"publisher","DOI":"10.1086\/317935"},{"key":"e_1_3_2_40_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.101130398"},{"key":"e_1_3_2_41_1","doi-asserted-by":"publisher","DOI":"10.1099\/vir.0.79889-0"},{"key":"e_1_3_2_42_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00335-004-3041-5"},{"key":"e_1_3_2_43_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1750-3639.1998.tb00165.x"},{"key":"e_1_3_2_44_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ejhg.5201544"},{"key":"e_1_3_2_45_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1083320"},{"key":"e_1_3_2_46_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awl226"},{"key":"e_1_3_2_47_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000267642.41594.9d"},{"key":"e_1_3_2_48_1","doi-asserted-by":"publisher","DOI":"10.1038\/352340a0"},{"key":"e_1_3_2_49_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410390613"},{"key":"e_1_3_2_50_1","doi-asserted-by":"publisher","DOI":"10.1038\/386232a0"},{"key":"e_1_3_2_51_1","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W"},{"key":"e_1_3_2_52_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.97.18.10168"},{"key":"e_1_3_2_53_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh249"},{"key":"e_1_3_2_54_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.6801762"},{"key":"e_1_3_2_55_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.95.23.13363"},{"key":"e_1_3_2_56_1","doi-asserted-by":"publisher","DOI":"10.1038\/2654"},{"key":"e_1_3_2_57_1","doi-asserted-by":"publisher","DOI":"10.1006\/geno.2000.6320"},{"key":"e_1_3_2_58_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.274.5295.2079"},{"key":"e_1_3_2_59_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2007.02.010"},{"key":"e_1_3_2_60_1","doi-asserted-by":"publisher","DOI":"10.1038\/9030"},{"key":"e_1_3_2_61_1","doi-asserted-by":"publisher","DOI":"10.1093\/bmb\/66.1.241"},{"key":"e_1_3_2_62_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1103932"},{"key":"e_1_3_2_63_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awl076"},{"key":"e_1_3_2_64_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0800190105"},{"key":"e_1_3_2_65_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrmicro1025"},{"key":"e_1_3_2_66_1","doi-asserted-by":"publisher","DOI":"10.1093\/bmb\/66.1.255"},{"key":"e_1_3_2_67_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004390050204"}],"container-title":["Philosophical Transactions of the Royal Society B: Biological Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/royalsocietypublishing.org\/doi\/pdf\/10.1098\/rstb.2008.0069","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/royalsocietypublishing.org\/doi\/full-xml\/10.1098\/rstb.2008.0069","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/royalsocietypublishing.org\/doi\/pdf\/10.1098\/rstb.2008.0069","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T21:22:30Z","timestamp":1778620950000},"score":1,"resource":{"primary":{"URL":"http:\/\/royalsocietypublishing.org\/rstb\/article\/363\/1510\/3747-3753\/58537"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,11,27]]},"references-count":66,"journal-issue":{"issue":"1510","published-print":{"date-parts":[[2008,11,27]]}},"alternative-id":["10.1098\/rstb.2008.0069"],"URL":"https:\/\/doi.org\/10.1098\/rstb.2008.0069","relation":{},"ISSN":["0962-8436","1471-2970"],"issn-type":[{"value":"0962-8436","type":"print"},{"value":"1471-2970","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,11,27]]},"assertion":[{"value":"2008-11-27","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}