RESUMO
In this paper we examine the traditional mortuary rites of the South Fore people of Papua New Guinea using Robert Hertz's theory of secondary burial and the three mechanisms of mourning identified by Daniel Lagache. The ethnographic data that we obtained on South Fore interpretations of their own mortuary rites showed that all forms of corpse handling achieved the same end results through the process of secondary burial. Furthermore, the three mechanisms of mourning applied equally to all forms of corpse disposal and we found no evidence to support psychosexual interpretations of mortuary anthropophagy which emphasise aggression. South Fore interpretations of mortuary anthropophagy show that the dead were eaten out of love, and to protect the mourners from the painful emotion of witnessing the decomposition of the corpse. These findings affirm the relevance of the concepts of Hertz and Lagache to the universal human experiences of death and mourning.
RESUMO
Prion diseases are fatal neurodegenerative diseases of humans and animals caused by the misfolding and aggregation of prion protein (PrP). Mammalian prion diseases are under strong genetic control but few risk factors are known aside from the PrP gene locus (PRNP). No genome-wide association study (GWAS) has been done aside from a small sample of variant Creutzfeldt-Jakob disease (CJD). We conducted GWAS of sporadic CJD (sCJD), variant CJD (vCJD), iatrogenic CJD, inherited prion disease, kuru and resistance to kuru despite attendance at mortuary feasts. After quality control, we analysed 2000 samples and 6015 control individuals (provided by the Wellcome Trust Case Control Consortium and KORA-gen) for 491032-511862 SNPs in the European study. Association studies were done in each geographical and aetiological group followed by several combined analyses. The PRNP locus was highly associated with risk in all geographical and aetiological groups. This association was driven by the known coding variation at rs1799990 (PRNP codon 129). No non-PRNP loci achieved genome-wide significance in the meta-analysis of all human prion disease. SNPs at the ZBTB38-RASA2 locus were associated with CJD in the UK (rs295301, P = 3.13 × 10(-8); OR, 0.70) but these SNPs showed no replication evidence of association in German sCJD or in Papua New Guinea-based tests. A SNP in the CHN2 gene was associated with vCJD [P = 1.5 × 10(-7); odds ratio (OR), 2.36], but not in UK sCJD (P = 0.049; OR, 1.24), in German sCJD or in PNG groups. In the overall meta-analysis of CJD, 14 SNPs were associated (P < 10(-5); two at PRNP, three at ZBTB38-RASA2, nine at nine other independent non-PRNP loci), more than would be expected by chance. None of the loci recently identified as genome-wide significant in studies of other neurodegenerative diseases showed any clear evidence of association in prion diseases. Concerning common genetic variation, it is likely that the PRNP locus contains the only strong risk factors that act universally across human prion diseases. Our data are most consistent with several other risk loci of modest overall effects which will require further genetic association studies to provide definitive evidence.
Assuntos
Predisposição Genética para Doença , Estudo de Associação Genômica Ampla , Polimorfismo de Nucleotídeo Único , Doenças Priônicas/genética , Príons/genética , Estudos de Casos e Controles , Síndrome de Creutzfeldt-Jakob/genética , Resistência à Doença , Encefalopatia Espongiforme Bovina/genética , Feminino , Humanos , Kuru/genética , Proteínas de Neoplasias/genética , Proteínas Priônicas , Fatores de Risco , Proteínas Ativadoras de ras GTPase/genéticaRESUMO
This paper is part of a wider study to explain the historical spread and changing epidemiological patterns of kuru by analysing factors that affect the transmission of kuru. Part of the study has been to look at the mortuary feasts that were the means of transmission of the kuru agent. This paper shows the complexity of Fore eschatology, and the variations and contradictions of human behaviour in relation to mortuary rites and the transmission of kuru. It also confirms that oral ingestion was the primary route of inoculation though some cases of parenteral inoculation may have occurred. The exclusion of alternative routes of transmission is of importance owing to the dietary exposure of the UK and other populations to bovine spongiform encephalopathy prions.