Kuru is a fatal subacute neurodegenerative disease restricted to the Fore people and their immediate neighbours in the Eastern Highlands of Papua New Guinea (PNG). It was the first of the human prion diseases shown experimentally to be transmissible. Its natural route of transmission was oral, through the consumption by extended kin of infective brain and other material from dead patients at mortuary feasts. Research into kuru began in 1957 and, following the work of many investigators, by the mid-1980s the research responsibility had devolved entirely on the Papua New Guinea Institute of Medical Research.
Collaborative studies on kuru commenced in 1996, initially funded by Wellcome Trust support to Professor Collinge, and this research was then incorporated into the Unit’s programmes at its inception in 1998 and kuru field studies were expanded and developed. Kuru provides the principal experience of an epidemic human prion disease, and one that is now reaching its end. In addition to its intrinsic interest, it provides an opportunity to learn much of relevance to understanding variant CJD, also thought to be caused by dietary prion exposure. Indeed vCJD gave kuru a new global relevance. In particular the Unit has explored the remarkable range of possible incubations in orally acquired human prion infection and performed detailed studies of genetic susceptibility to acquired prion disease. These studies continue to be of major importance to the Unit’s genetic research and in understanding the role of prion strains and route of exposure in determining prion pathogenesis. These studies therefore are of major importance to the Unit’s mission both with respect to our fundamental studies of prion biology and to inform ongoing public health concerns posed by vCJD, BSE and other emerging, potentially zoonotic prion diseases. Kuru is also of wider importance to the history of medicine and human society, and the Unit is documenting cultural practices and traditions that will otherwise soon otherwise be lost. The interaction of the Unit with our collaborators at the PNG Institute of Medical Research, with the Fore and other communities affected by kuru and with Papua New Guinean society more generally has enormously enriched the life and work of the Unit and National Prion Clinic at many levels.
Further details of the Unit's work in PNG can be accessed via the link below.
HECTD2 is associated with susceptibility to mouse and human prion diseases.
Lloyd,SE, Maytham EG, Pota H, Grizenkova J, Molou E, Uphill J, Hummerich H, Whitfield J, Alpers MP, Mead S, and Collinge J.
PLoS Genetics 2009.
Genetic risk factors for variant Creutzfeldt-Jakob disease: a genome-wide association study
Mead S, Poulter M, Uphill J, Beck J, Whitfield J, Webb TE, Campbell T, Adamson G, Deriziotis P, Tabrizi SJ, Hummerich H, Verzilli C, Alpers MP, Whittaker JC, Collinge J
Lancet Neurol 2009;8(1):57-66.
Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease
Brandner S, Whitfield J, Boone K, Puwa A, O’Malley C, Linehan JM, Joiner S, Scaravilli F, Calder I, Alpers MP, Wadsworth JDF, Collinge J.
Phil Trans R Soc B 2008;363:3755-3763
The origin of the prion agent of kuru: molecular and biological strain typing.
Wadsworth JDF, Joiner S, Linehan JM, Asante EA, Brandner S, Collinge J.
Phil Trans R Soc B 2008;363:3747-3753
Genetic susceptibility, evolution and the kuru epidemic
Mead S, Whitfield J, Poulter M, Shah P, Uphill J, Beck J, Campbell T, Al-Dujaily H, Hummerich H, Alpers MP, Collinge J.
Phil Trans R Soc B 2008;363:3741-3746.
A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea
Collinge J, Whitfield J, McKintosh E, Frosh A, Mead S, Hill AF, Brandner S, Thomas D, Alpers MP.
Phil Trans R Soc B 2008;363:3725-3739.
Mortuary rites of the South Fore and kuru
Whitfield JT, Pako WH, Collinge J, Alpers MP.
Phil Trans R Soc B 2008;363:3721-3724
The epidemiology of kuru: monitoring the epidemic from its peak to its end
Phil Trans R Soc B 2008;363:3707-3713
Lessons of kuru research: background to recent studies with some personal reflections
Phil Trans R Soc B 2008;363:3689-3696
Work among the people of the Okapa area from 1996 to the present
Phil Trans R Soc B 2008;363:3671-3672
'Most people still believe that kuru is caused by sorcery’
Phil Trans R Soc B 2008;363:3656
The work of the Kuru Field Unit, Kuru Research Project of the Papua New Guinea Institute of Medical Research and MRC Prion Unit.
Phil Trans R Soc B 2008;363:3652
An account of the last autopsy carried out on a kuru patient
Phil Trans R Soc B 2008;363:3630
Some tributes to research colleagues and other contributors to our knowledge about kuru
Phil Trans R Soc B 2008;363:3614-3617
Reminiscences and reflections on kuru, personal and scientific
Collinge J, Alpers MP.
Phil Trans R Soc B 2008;363:3613-3673
Introduction to ‘The end of kuru: 50 years of research into an extraordinary disease’
Collinge J, Alpers MP.
Phil Trans R Soc B 2008;363:3607-3612
The End of Kuru: 50 Years of Research into an Extraordinary Disease
Collinge J, Alpers MP, eds.
A Theme Issue of the Philosophical Transactions of the Royal Society B 2008 Nov 27;363(1510):3605-3763
Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice
Wadsworth JDF, Joiner S, Linehan JM, Desbruslais M, Fox K, Cooper S, Cronier S, Asante EA, Mead S, Brandner S, Hill AF, Collinge J.
Proc Natl Acad Sci USA 2008;105:3885-3890
A history of kuru.
PNG Med J 2007;50:10-19
Kuru in the 21st century – an acquired human prion disease with very long incubation periods
Collinge J, Whitfield J, McKintosh E, Beck J, Mead S, Thomas DJ, Alpers MP.
The epidemiology of kuru in the period 1987 to 1995.
Alpers MP, Kuru Surveillance Team.
Commun Dis Intell 2005;29:391-399
Variant Creutzfeldt-Jakob disease: implications for the health care system
Dunstan RA, Alpers MP.
Aust NZ J Public Health 2005;29:308-312
Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics
Mead S, Stumpf MPH, Whitfield J, Beck JA, Poulter M, Campbell T, Uphill JB, Goldstein D, Alpers MP, Fisher EMC, Collinge J.