A5006250755- Prion Diseases and Protein Misfolding
- Neurological diseases and metabolism
- Trace Elements in Health
- Folate and B Vitamins Research
- Rangeland and Wildlife Management
- Brucella: diagnosis, epidemiology, treatment
- Healthcare Systems and Challenges
- Ecology and Vegetation Dynamics Studies
- Metabolism and Genetic Disorders
- Archaeology and ancient environmental studies
- Health Services Management and Policy
- Lepidoptera: Biology and Taxonomy
- Infectious Encephalopathies and Encephalitis
- Amino Acid Enzymes and Metabolism
- Birth, Development, and Health
- Paleopathology and ancient diseases
- Amazonian Archaeology and Ethnohistory
- Genetic diversity and population structure
- Ancient Mediterranean Archaeology and History
- Grief, Bereavement, and Mental Health
- Livestock and Poultry Management
- Anthropological Studies and Insights
- Diet and metabolism studies
- Neurological and metabolic disorders
- Pacific and Southeast Asian Studies
MRC Prion Unit
2003-2024
University College London
2003-2024
Medical Research Council
2003-2020
University College London Hospitals NHS Foundation Trust
2020
National Hospital for Neurology and Neurosurgery
2003-2017
Papua New Guinea Institute of Medical Research
2003-2015
Curtin University
2003-2015
Royal College of Physicians
1970
Kuru is an acquired prion disease largely restricted to the Fore linguistic group of Papua New Guinea Highlands, which was transmitted during endocannibalistic feasts. Heterozygosity for a common polymorphism in human protein gene ( PRNP ) confers relative resistance diseases. Elderly survivors kuru epidemic, who had multiple exposures at mortuary feasts, are, marked contrast younger unexposed Fore, predominantly 129 heterozygotes. imposed strong balancing selection on essentially...
Kuru is a devastating epidemic prion disease that affected highly restricted geographic area of the Papua New Guinea highlands; at its peak, it predominantly adult women and children both sexes. Its incidence has steadily declined since cessation route transmission, endocannibalism.
Human and animal prion diseases are under genetic control, but apart from PRNP (the gene that encodes the protein), we understand little about human susceptibility to bovine spongiform encephalopathy (BSE) prions, causal agent of variant Creutzfeldt-Jakob disease (vCJD).We did a genome-wide association study risk vCJD tested for replication our findings in samples many categories (929 samples) control UK Papua New Guinea (4254 samples), including controls who were genotyped by Wellcome Trust...
Kuru is so far the principal human epidemic prion disease. While its incidence has steadily declined since cessation of route transmission, endocannibalism, in Papua New Guinea 1950s, arrival variant Creutzfeldt-Jakob disease (vCJD), also thought to be transmitted by dietary exposure, given kuru a new global relevance. We investigated all suspected cases from July 1996 June 2004 and identified 11 patients. There were four females seven males, with an age range 46-63 years at onset disease,...
While the neuropathology of kuru is well defined, there are few data concerning distribution disease-related prion protein in peripheral tissues. Here we report investigation brain and tissues from a patient who died 2003. Neuropathological findings were compared with those seen classical (sporadic iatrogenic) Creutzfeldt–Jakob disease (CJD) variant CJD (vCJD). The neuropathological showed all stereotypical changes that define kuru, occurrence prominent PrP plaques throughout brain....
Prion diseases are fatal transmissible neurodegenerative disorders, which include Scrapie, Bovine Spongiform Encephalopathy (BSE), Creutzfeldt-Jakob Disease (CJD), and kuru. They characterised by a prolonged clinically silent incubation period, variation in is determined many factors, including genetic background. We have used heterogeneous stock of mice to identify Hectd2, an E3 ubiquitin ligase, as quantitative trait gene for prion disease time mice. Further, we report association between...
The acquired prion disease kuru was restricted to the Fore and neighbouring linguistic groups of Papua New Guinea highlands largely affected children adult women. Oral history documents onset epidemic in early twentieth century, followed by a peak mid-twentieth century subsequently well-documented decline frequency. In context these strong associations (gender, region time), we have considered genetic factors associated with susceptibility resistance kuru. Heterozygosity at codon 129 human...
This paper is part of a wider study to explain the historical spread and changing epidemiological patterns kuru by analysing factors that affect transmission kuru. Part has been look at mortuary feasts were means agent. shows complexity Fore eschatology, variations contradictions human behaviour in relation rites It also confirms oral ingestion was primary route inoculation though some cases parenteral may have occurred. The exclusion alternative routes importance owing dietary exposure UK...
Kuru is a prion disease which became epidemic among the Fore and surrounding linguistic groups in Papua New Guinea, peaking late 1950s. It was transmitted during transumption (endocannibalism) of dead family members at mortuary feasts. In this study, we aimed to explain historical spread changing epidemiological patterns kuru by analysing factors that affected its transmission. We also examined what cultural group principally determined family's behaviour rituals. Our investigations showed...
Ethnographic studies on Melanesian concepts of the human body and religion have expanded our understandings concept personhood. ethnographers used a number descriptive words to describe metaphysical components person, including: soul, spirit, life force, ghost, vital spiritual essence. By investigating traditional mortuary rites South Fore people in Papua New Guinea, which included practice endocannibalism, investigators were able distinguish 5 souls composite person their relationship...
I have conducted fieldwork on kuru, with colleagues from the MRC Prion Unit and Papua New Guinea Institute of Medical Research (PNGIMR), since 1996. During this time, has been dependent support PNGIMR, thank staff past present Directors, Michael Alpers, John Reeder Peter Siba, for their support. Michael's vast knowledge experience invaluable inspirational. also my Guinean friends whose counsel guided me through occasional turbulent time. In particular, Dr Inoni Betuela, Ken Boone his wife...
Abstract Mammalian prions are lethal pathogens composed of fibrillar assemblies misfolded prion protein. Human diseases rare and usually rapidly fatal neurodegenerative disorders, the most common being sporadic Creutzfeldt-Jakob disease (sCJD). Variants in gene that encodes protein ( PRNP ) strong risk factors for sCJD, but although condition has heritability similar to other no loci have yet been confirmed. By genome-wide association European ancestry populations, we found three replicated...
In this paper we examine the traditional mortuary rites of South Fore people Papua New Guinea using Robert Hertz’s theory secondary burial and three mechanisms mourning identified by Daniel Lagache. The ethnographic data that obtained on interpretations their own showed all forms corpse handling achieved same end results through process burial. Furthermore, applied equally to disposal found no evidence support psychosexual anthropophagy which emphasise aggression. show dead were eaten out...
Populations of the Eastern Highlands Papua New Guinea (EHPNG, area 11,157 km2) lived in relative isolation from rest world until mid-20th century, and region contains a wealth linguistic cultural diversity. Notably, several populations EHPNG were devastated by an epidemic prion disease, kuru, which at its peak mid-twentieth century led to some villages being almost depleted adult women. Until now, population genetic analyses learn about diversity, migration, admixture, impact kuru have been...