Review. Lessons of kuru research: Background to recent studies with some personal reflections

Philosophical Transactions of the Royal Society B: Biological Sciences

Volumn 363, Issue 1510, 2008, Pages 3689-3696

  Collinge, John ^a  

^a NATIONAL HOSPITAL FOR NEUROLOGY AND NEUROSURGERY   (United Kingdom)

Author keywords

Kuru; Prion; Public health

Indexed keywords

BOVINE SPONGIFORM ENCEPHALOPATHY; DISEASE TRANSMISSION; EPIDEMIC; INCUBATION; INFECTIOUS DISEASE; PATHOLOGY; PRION DISEASE; PUBLIC HEALTH; RESEARCH;

MELANESIA; PACIFIC ISLANDS; PACIFIC OCEAN; PAPUA NEW GUINEA;

BOVINAE;

EID: 53749097930     PISSN: 09628436     EISSN: None     Source Type: Journal    
DOI: 10.1098/rstb.2008.0121     Document Type: Conference Paper

References (34)

1. Alpers, M. P. 2008 The epidemiology of kuru: monitoring the epidemic from its peak to its end. Phil. Trans. R. Soc. B 363, 3707-3713. (doi:10.1098/rstb.2008.0071)
2. Alpers, M. & Rail, L. 1971 Kuru and Creutzfeldt-Jakob disease: clinical and aetiological aspects. Proc. Aust. Assoc. Neurol. 8, 7-15.
3. Bateman, D., Hilton, D., Love, S., Zeidler, M., Beck, J. & Collinge, J. 1995 Sporadic Creutzfeldt-Jakob disease in a 18-year old in the UK. Lancet 346, 1155-1156. (doi:10.1016/S0140-6736(95)91828-0)
4. Brandner, S. et al. 2008 Central and peripheral pathology of kuru: pathological analysis of a recent case and comparision with other forms of human prion disease. Phil. Trans. R. Soc. B 363, 3755-3763. (doi:10.1098/rstb.2008.0091)
5. Britton, T. C., Al-Sarraj, S., Shaw, C., Campbell, T. & Collinge, J. 1995 Sporadic Creutzfeldt-Jakob disease in a 16-year-old in the UK. Lancet 346, 1155. (doi:10.1016/S0140-6736(95)91827-2)
6. Brown, P., Cathala, F., Raubertas, R. F., Gajdusek, D. C. & 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, 895-904.
7. Brown, P., Brandel, J. P., Preese, M. & Sato, T. 2006 Iatrogenic Creutzfeldt-Jakob disease. The waning of an era. Neurology 67, 389-393. (doi:10.1212/01.wnl.0000231528.65069.3f)
8. Bruce, M. E. et al. 1997 Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature 389, 498-501. (doi:10.1038/39057)
9. Collinge, J. & Clarke, A. 2007 A general model of prion strains and their pathogenicity. Science 318, 930-936. (doi:10.1126/science. 1138718)
10. Collinge, J., Harding, A. E., Owen, F., Poulter, M., Lofthouse, R., Boughey, A. M., Shah, T. & Crow, T. J. 1989 Diagnosis of Gerstmann-Straussler syndrome in familial dementia with prion protein gene analysis. Lancet 2, 15-17. (doi:10.1016/S0140-6736(89)90256-0)
11. Collinge, J. et al. 1990 Prion dementia without characteristic pathology. Lancet 336, 7-9. (doi:10.1016/0140-6736(90)91518-F)
12. Collinge, J. et al. 1995 Unaltered susceptibility to BSE in transgenic mice expressing human prion protein. Nature 378, 779-783. (doi:10.1038/378779a0)
13. Collinge, J., Sidle, K. C. L., Meads, J., Ironside, J. & Hill, A. F. 1996 Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature 383, 685-690. (doi:10.1038/383685a0)
14. Collinge, J., Whitfield, J., McKintosh, E., Beck, J., Mead, S., Thomas, D. J. & Alpers, M. 2006 Kuru in the 21st century-an acquired human prion disease with very long incubation periods. Lancet 367, 2068-2074. (doi:10.1016/S0140-6736(06)68930-7)
15. Collinge, J., Whitfield, J., McKintosh, E., Frosh, A., Mead, S., Hill, A. F., Brandner, S., Thomas, D. & Alpers, M. P. 2008 A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea. Phil. Trans. R. Soc. B 363, 3725-3739. (doi:10.1098/rstb.2008.0068)
16. Gajdusek, D. C., Gibbs Jr, C. J. & Alpers, M. 1966 Experimental transmission of a kuru-like syndrome to chimpanzees. Nature 209, 794-796. (doi:10.1038/209794a0)
17. Ghani, A. C., Donnelly, C. A., Ferguson, N. M. & Anderson, R. M. 2002 The transmission dynamics of BSE and vCJD. CR Acad. Sci. III 325, 37-47.
18. Griffith, J. S. 1967 Self replication and scrapie. Nature 215, 1043-1044. (doi:10.1038/2151043a0)
19. Hill, A. F., Desbruslais, M., Joiner, S., Sidle, K. C. L., Gowland, I. & Collinge, J. 1997 The same prion strain causes vCJD and BSE. Nature 389, 448-450. (doi:10.1038/38925)
20. Hsiao, K., Baker, H. F., Crow, T. J., Poulter, M., Owen, F., Terwilliger, J. D., Westaway, D., Ott, J. & Prusiner, S. B. 1989 Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome. Nature 338, 342-345. (doi:10.1038/338342a0)
21. Mead, S. et al. 2003 Balancing selection at the prion protein gene consistent with prehistoric kuru-like epidemics. Science 300, 640-643. (doi:10.1126/science.1083320)
22. Mead, S. et al. 2008 Genetic susceptibility, evolution and the kuru epidemic. Phil. Trans. R. Soc. B 363, 3741-3746. (doi:10.1098/rstb.2008.0087)
23. Owen, F. et al. 1989 Insertion in prion protein gene in familial Creutzfeldt-Jakob disease. Lancet 1, 51-52. (doi:10.1016/S0140- 6736(89)91713-3)
24. Prusiner, S. B. 1982 Novel proteinaceous infectious particles cause scrapie. Science 216, 136-144. (doi:10.1126/science.6801762)
25. Prusiner, S. B. et al. 1990 Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell 63, 673-686. (doi:10.1016/0092-8674(90)90134-Z)
26. Tabrizi, S., Scaravilli, F., Howard, R. S., Collinge, J. & Rossor, M. N. 1996 Creutzfeldt-Jakob disease in a young woman. Report of a meeting of physicians and scientists, St. Thomas' Hospital, London. Lancet 347, 945-948. (doi:10.1016/S0140-6736(96)91419-1)
27. Wadsworth, J. D. et al. 2008a Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice. Proc. Natl Acad. Sci. USA 105, 3885-3890. (doi:10.1073/pnas.0800190105)
28. Wadsworth, J. D. F., Joiner, S., Linehan, J. M., Asante, E. A., Brandner, S. & Collinge, J. 2008b The origin of the prion agent of kuru: molecular and biological strain typing. Phil. Trans. R. Soc. B 363, 3747-3753. (doi:10.1098/rstb.2008.0069)
29. Wells, G. A. H., Scott, A. C., Johnson, C. T., Gunning, R. F., Hancock, R. D., Jeffrey, M., Dawson, M. & Bradley, R. 1987 A novel progressive spongiform encephalopathy in cattle. Vet. Rec. 31, 419-420.
30. Whitfield, J. T., Pako, W. H., Collinge, J. & Alpers, M. P. 2008 Mortuary rites of the South Fore and kuru. Phil. Trans. R. Soc. B 363, 3721-3724. (doi:10.1098/rstb.2008.0074)
31. Wickner, R. B., Edskes, H. K., Ross, E. D., Pierce, M. M., Baxa, U., Brachmann, A. & Shewmaker, F. 2004 Prion genetics: new rules for a new kind of gene 12. Annu. Rev. Genet. 38, 681-707. (doi:10.1146/annurev. genet.38.072902.092200)
32. Will, R. G. et al. 1996 A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 347, 921-925. (doi:10.1016/S0140-6736(96)91412- 9)
33. Wroe, S. J. et al. 2006 Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report. Lancet 368, 2061-2067. (doi:10.1016/S0140-6736(06)69835-8)
34. Wyatt, J. M., Pearson, G. R., Smerdon, T. N., Gruffydd-Jones, T. J., Wells, G. A. H. & Wilesmith, J. W. 1991 Naturally occurring scrapie-like spongiform encephalopathy in five domestic cats. Vet. Rec. 129, 233-236.