Search

This essay interrogates the intersections of colonialism, western science, and desire through an analysis of the outbreak of kuru in Papua New Guinea in the mid-20th century. I argue that cannibalism drew eyes to the eastern highlands, and the discovery of kuru kept the colonial gaze fixed firmly on the region. Finally, I explore how the appetites of D. Carleton Gajdusek, both medical and sexual, further entrenched western power. [ABSTRACT FROM AUTHOR]

Populations of the Eastern Highlands of Papua New Guinea (EHPNG, area 11,157 km2) lived in relative isolation from the rest of the world until the mid-20th century, and the region contains a wealth of linguistic and cultural diversity. Notably, several populations of EHPNG were devastated by an epidemic prion disease, kuru, which at its peak in the mid-twentieth century led to some villages being almost depleted of adult women. Until now, population genetic analyses to learn about genetic diversity, migration, admixture, and the impact of the kuru epidemic have been restricted to a small number of variants or samples. Here, we present a population genetic analysis of the region based on genome-wide genotype data of 943 individuals from 21 linguistic groups and 68 villages in EHPNG, including 34 villages in the South Fore linguistic group, the group most affected by kuru. We find a striking degree of genetic population structure in the relatively small region (average F ST between linguistic groups 0.024). The genetic population structure correlates well with linguistic grouping, with some noticeable exceptions that reflect the clan system of community organization that has historically existed in EHPNG. We also detect the presence of migrant individuals within the EHPNG region and observe a significant excess of females among migrants compared to among non-migrants in areas of high kuru exposure (p = 0.0145, chi-squared test). This likely reflects the continued practice of patrilocality despite documented fears and strains placed on communities as a result of kuru and its associated skew in female incidence. Explore an in-depth investigation of the Eastern Highlands of Papua New Guinea. Analyses attempt to disentangle the complex relationships between genetics, linguistics, and the clan system of political organization. Additionally, an analysis investigating the impact of the kuru epidemic on migration flows in the region is undertaken. [ABSTRACT FROM AUTHOR]

Copyright of Neurologie Pro Praxi is the property of SOLEN sro and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Neurologie Pro Praxi is the property of SOLEN sro and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Prion diseases and the prion protein are only partially understood so far in many aspects. This explains the continued research on this topic, calling for an overview on the current state of knowledge. The main objective of the present review article is to provide a comprehensive up-to-date presentation of all major features of human prion diseases bridging the gap between basic research and clinical aspects. Starting with the prion protein, current insights concerning its physiological functions and the process of pathological conversion will be highlighted. Diagnostic, molecular, and clinical aspects of all human prion diseases will be discussed, including information concerning rare diseases like prion-associated amyloidoses and Huntington disease-like 1, as well as the question about a potential human threat due to the transmission of prions from prion diseases of other species such as chronic wasting disease. Finally, recent attempts to develop future therapeutic strategies will be addressed.

Copyright of Journal of Soil Science & Plant Nutrition / Toprak Bilimi ve Bitki Besleme Dergisi is the property of Soil Science Society of Turkey and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Les maladies à prions ou encéphalopathies spongiformes transmissibles (EST) sont des maladies humaines et animales transmissibles naturellement ou expérimentalement avec une longue durée d'incubation et une évolution fatale sans rémission. La nature exacte de l'agent transmissible reste discutée mais l'absence de structure évoquant un micro-organisme conventionnel a conduit Stanley B. Prusiner à émettre l'hypothèse qu'il pourrait s'agir d'une protéine infectieuse (proteinaceous infectious particle ou prion). Le prion serait la forme anormale d'une protéine normale, la PrP cellulaire (PrPc) qui va changer de conformation spatiale et être convertie en protéine prion scrapie (PrPsc) ayant des propriétés de résistance partielle aux protéases, d'agrégation et d'insolubilité dans les détergents. Les EST ne s'accompagnent d'aucune réaction inflammatoire ou immunitaire et se caractérisent par les lésions cérébrales associant spongiose, raréfaction neuronale, gliose astrocytaire et accumulation de la PrPsc pouvant prendre l'aspect de plaques amyloïdes. Si le lien entre l'accumulation de PrPsc et la survenue des lésions est discuté, la présence de PrPsc est constante au cours des EST et sa mise en évidence est nécessaire au diagnostic de certitude. Même si elles restent des maladies rares (2 cas par million), l'identification du kuru, à la fin des années 1950, des cas iatrogènes au courant des années 1970 et de la variante de la maladie de Creutzfeldt-Jakob (MCJ) au milieu des années 1990 expliquent l'intérêt pour ces maladies, mais aussi les craintes qu'elles peuvent faire peser sur la santé publique. Elles demeurent un modèle passionnant de recherche car elles appartiennent à la fois au groupe des maladies neurodégénératives avec accumulation de protéines (MCJ sporadique), au groupe des maladies transmissibles (MCJ iatrogènes, variante de la MCJ) mais aussi au groupe des affections génétiques de transmission mendélienne dominante (MCJ génétiques, syndrome de Gerstmann-Straussler-Scheinker, insomnie fatale familiale). Prion diseases or transmissible spongiform encephalopathies (TSEs) are human and animal diseases naturally or experimentally transmissible with a long incubation period and a fatal course without remission. The nature of the transmissible agent remains debated but the absence of a structure evoking a conventional microorganism led Stanley B. Prusiner to hypothesize that it could be an infectious protein (proteinaceous infectious particle or prion). The prion would be the abnormal form of a normal protein, cellular PrP (PrPc) which will change its spatial conformation and be converted into scrapie prion protein (PrPsc) with properties of partial resistance to proteases, aggregation and insolubility in detergents. No inflammatory or immune response are detected in TSEs which are characterized by brain damage combining spongiosis, neuronal loss, astrocytic gliosis, and deposits of PrPsc that may appear as amyloid plaques. Although the link between the accumulation of PrPsc and the appearance of lesions remains debated, the presence of PrPsc is constant during TSE and necessary for a definitive diagnosis. Even if they remain rare diseases (2 cases per million), the identification of kuru, at the end of the 1950s, of iatrogenic cases in the course of the 1970s and of the variant of Creutzfeldt-Jakob disease (CJD) in the mid-1990s explain the interest in these diseases but also the fears they can raise for public health. They remain an exciting research model because they belong both to the group of neurodegenerative diseases with protein accumulation (sporadic CJD), to the group of communicable diseases (iatrogenic CJD, variant of CJD) but also to the group of genetic diseases with a transmission Mendelian dominant (genetic CJD, Gerstmann-Straussler-Scheinker syndrome, fatal familial insomnia). [ABSTRACT FROM AUTHOR]

A discussion of whiteness as an "ethos" or "relational category" in bioethics, drawing on examples from medical and historical research. [ABSTRACT FROM AUTHOR]

This is a reflection on the close relations of the writing of postcolonial histories and recent decolonial critiques, and on the tensions between them. Postcolonial historical analysis often has been preoccupied with hybridity and mixture, conjugation and adaptation, exchange and interaction—with subversions of sovereignty in contact zones, borderlands, and on the beach. As a structuralist formulation, decolonial historical binarism in contrast echoes Indigenous politics of self‐determination, even suggesting at times an ontological decoupling of settler and Indigenous histories and practices. Stringent decolonization of historical inquiry—implying the sabotage and superseding of settler colonial linguistic, narrative, and temporal conventions and the disturbing of standardized assumptions about evidence, agency, and authorship—would give us an epistemic assemblage perhaps not recognizable as "history." Even if desirable, is that imaginable now except as metaphor or ideal? [ABSTRACT FROM AUTHOR]

Objectives: The island of New Guinea was settled by modern human over 50,000 years ago, and is currently characterized by a complex landscape and contains one‐seventh of the world's languages. The Eastern Highlands of New Guinea were also the home to the devastating prion disease called kuru that primarily affected Fore‐speaking populations, with some 68% of cases involving adult females. We characterized the mitochondrial DNA (mtDNA) diversity of highlanders from Papua New Guinea (PNG) to: (a) gain insight into the coevolution of genes and languages in situ over mountainous landscapes; and (b) evaluate the recent influence of kuru mortality on the pattern of female gene flow. Materials and Methods: We sequenced the mtDNA hypervariable segment 1 of 870 individuals from the Eastern and Southern Highlands of PNG using serums collected in the 1950s to 1960s. These highlanders were selected from villages representing 15 linguistic groups within the Trans‐New Guinea phylum. Genetic, linguistic, and geographic distances were calculated separately and correlations among those distance matrices were assessed using the Mantel test. Results: Geographic, genetic, and linguistic patterns were independently correlated with each other (p <.05). Increased mtDNA diversity in kuru‐affected populations and low Fst estimates between kuru‐affected linguistic groups were observed. Discussion: In general, the genetic structure among the Highland populations was shaped by both geography and language, and language is a good predictor of mtDNA affinity in the PNG Highlands. High kuru female mortality increased female gene flow locally, disrupting coevolutionary pattern among genes, languages, and geography. [ABSTRACT FROM AUTHOR]

Prion diseases are a phenotypically diverse set of disorders characterized by protease-resistant abnormally shaped proteins known as prions. There are three main groups of prion diseases, termed sporadic (Creutzfeldt-Jakob disease [CJD], sporadic fatal insomnia, and variably protease-sensitive phonopathy), genetic (genetic CJD, fatal familial insomnia, and Gerstmann-Straussler-Scheinker syndrome), and acquired (kuru, variant CJD, and iatrogenic CJD). This article will review the pathophysiology, genetics, clinical presentations, and diagnostic challenges in patients with prion disease. Case discussions, images, and tables will be used to highlight important characteristics of prion disease and prion mimics. [ABSTRACT FROM AUTHOR]

Shirley Lindenbaum’s study in the early 1960s of the origins and transmission of kuru among the Fore people of the eastern highlands of New Guinea is one of the earliest examples of an explicitly medical anthropology. Lindenbaum later described her investigations as assembling ‘an epidemiology of social relations’. How might the emergence of medical anthropology, then, be related to the concurrent development of the social history of medicine and global epidemic intelligence? Are these alternative genealogies for medical anthropology?

The MV1 and MV2 subtypes of sporadic Creutzfeldt-Jakob disease (sCJD) are linked to the heterozygous methionine (M)/valine (V) polymorphism at codon 129 of the prion protein (PrP) gene. MV2 is phenotypically heterogeneous, whereas MV1, due to its low prevalence, is one of the least well characterized subtypes. In this study, we investigated the biochemical properties of PrP Sc and phenotypic expression of cases diagnosed as sCJD MV1 and MV2. We describe four MV2 histotypes: 2C, with cortical (C) coarse pathology; 2K, with kuru (K) plaque deposits; 2C-K, with co-existing C and K histotypic features; and the novel histotype 2C-PL that mimics 2C in the cerebral cortex and cerebellum, but exhibits plaque-like (PL) PrP deposits in subcortical regions (e.g., basal nuclei, thalamus and midbrain). Histotype prevalence is highest for 2C-K (55%), intermediate for 2C (31%), and lowest for 2C-PL and 2K (7%). Nearly every MV2 case expressed both PrP Sc types, with T2 being the predominant type ("MV2-1"). MV1 cases typically show a rapid disease course (≤ 4 months), and feature the 1C histotype, phenotypically identical to sCJDMM1. Co-existing PrP Sc types, with T1 significantly exceeding T2 ("MV1-2"), are detected in patients diagnosed as MV1 with longer disease courses. We observed four histotypes among MV1-2 cases, including two novel histotypes: 1V, reminiscent of sCJDVV1; 1C-2C, resembling sCJDMM1-2 with predominant MM1 histotypic component; and novel histotypes 1C-2PL and 1C-2K, overall mimicking 1C in the cerebral cortex, but harboring T2 and plaque-like PrP deposits in subcortical regions (1C-2PL), and T2 and kuru plaques in the cerebellum (1C-2K). Lesion profiles of 1C, 1V, and 1C-2C are similar, but differ from 1C-2PL and 1C-2K, as the latter two groups show prominent hippocampal and nigral degeneration. We believe that the novel "C-PL" histotypes are distinct entities rather than intermediate forms between "C" and "C-K" groups, and that 1C-2PL and 1C-2K histotypes may be characterized by different T1 variants of the same size., (© 2023. BioMed Central Ltd., part of Springer Nature.)