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A coronal section through the kuru-affected brain. 68-3538-10. Courtesy of late D. Carleton Gajdusek.
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Kuru, the first human transmissible spongiform encephalopathy was transmitted to chimpanzees by D. Carleton Gajdusek (1923-2008). In this review, I briefly summarize the history of this seminal discovery along its epidemiology, clinical picture, neuropathology and molecular genetics. The discovery of kuru opened new windows into the realms of human...
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The transformation of cellular prion protein (PrPc) into pathogenic conformer (PrPSc) in transmissible spongiform encephalopathy is expedited by mutations in the prion protein. One recently reported novel mutation V176G is located in region of the protein known to cause Creutzfeldt-Jakob disease (CJD) but possess a unique neuropathological profile...
Human prion disorders (transmissible spongiform encephalopathies, TSEs) are unique, progressive, and fatal neurodegenerative diseases caused by aggregation of misfolded prion protein in neuronal tissue. Due to the potential transmission, human TSEs are under active surveillance in a majority of countries; in the Czech Republic data are centralized...
Prions are transmissible agents causing lethal neurodegenerative diseases that are composed of aggregates of misfolded cellular prion protein (PrPSc). Despite non-fibrillar oligomers having been proposed as the most infectious prion particles, prions purified from diseased brains usually consist of large and fibrillar PrPSc aggregates, whose protea...
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... Placas amiloides unicêntricas foram também observadas em 50 a 75% dos cérebros examinados. Atualmente, a doença é considerada extinta (Liberski, 2013;Ladogana et al., 2018;Liberski et al., 2019). ...
As doenças priônicas (DPr) humanos são um grupo de doenças neurodegenerativas progressivas, incuráveis e fatais causadas por um agente infeccioso proteico (PrP), capaz de propagar a doença alterando a estrutura conformacional de proteínas, as quais sofrem agregação e depósito no tecido neuronal. Devido ao potencial neurodegenerativo desta doença, bem como a um aumento global de casos, o objetivo do nosso artigo é revisar o conhecimento atual sobre DPr humanas e analisar a situação epidemiológica dessas doenças, no Brasil nos últimos 17 anos. Sabemos, que existem diferentes tipos de DPr com diferenças relacionadas a sua forma de transmissão/manifestação, neuropatologia e manifestações clínicas e que os príons consistem em PrPSc a forma patológica agregada da proteína priônica celular PrPc. Apesar dos mecanismos envolvidos na neurodegeneração não estarem totalmente descritos, sabemos que envolvem múltiplos processos operando simultânea e sinergicamente no cérebro, incluindo degeneração espongiforme, alterações sinápticas, inflamação cerebral, morte neuronal e acúmulo de agregados proteicos. No Brasil, as DPr tornaram-se doenças de notificação compulsória ao Sistema de Informação de Agravos de Notificação (SINAN) a partir de 2005, sendo confirmados até 2020 mais de 400 casos de DPr, com aumento significativo de casos a partir do ano de 2012, principalmente nos estados de São Paulo, Minas Gerais e Paraná, provavelmente pela maior capacidade de diagnóstico destes estados. Dessa forma, uma compreensão mais abrangente destas doenças e sua epidemiologia pode ajudar no diagnóstico precoce e desenvolvimento de terapias muito necessárias para estas doenças devastadoras.
... Kuru, now an extinct disease, was endemic in Papua New Guinea for much of the twentieth century. is neurodegenerative disease caused by prions is characterized by ataxia and emotional alterations and wasting leading invariably to death within two years from symptom onset and has a long incubation period of up to more than 50 years [80][81][82]. e disease was related to ritualistic transumption in which the decedents' bodies (including the infected brain) were consumed by mourners. With the prohibition of ritualistic transumption in the 1950s, the number of deaths from Kuru declined sharply, with last Kuru related death reported in 2005 [82,83]. ...
Rituals are an integral part of human life but a wide range of rituals (both religious and non-religious), from self-flagellation to blood brotherhood to ritual sprinkling of holy water, have been associated with transmission of infections. These infections include angiostrongyliasis, anthrax, brucellosis, cholera, COVID-19, cutaneous larva migrans, Ebola, hepatitis viruses, herpes simplex virus, HIV, human T-cell leukemia virus (HTLV), kuru, Mycobacterium bovis, Naegleria fowleri meningoencephalitis, orf, rift valley fever, and sporotrichosis. Education and community engagement are important cornerstones in mitigating infectious risks associated with rituals.
... 3.6 Amyloid-like structures in embryonic cuticle are unable to accelerate amyloid formation under inflammatory conditions Some of the amyloid disorders in humans are infectious in nature. For example, prion diseases such as Kuru is propagated due to consumption of brain from a dead infected person as seen in the native people of Papua New Guinea (Liberski 2013). Several studies with animal models of amyloid A (AA) amyloidosis also showed that consumption of food containing amyloid fibres can induce severe amyloidosis and reduce the duration of induction in susceptible animals (an inflammatory condition) by seeding phenomenon (Lundmark et al. 2002;Solomon et al. 2007). ...
Artemia cysts are the essential food product for industrial larviculture of fishes. The cyst shell protects the artemia embryo from mechanical damage, ultraviolet light, excessive water loss, thermal variation and anoxia condition. However, the underlying mechanism of such environmental protection is largely unclear. The embryonic cuticle of cyst shell mainly constitutes chitin and proteins. Absence of cyst shell proteins compromises embryo survival. In literature, there are few examples of functional amyloids where proteins adapt amyloid-like structures and act as protective covering. We hypothesized that the proteins from the embryonic cuticle of artemia cyst shell may have amyloid-like properties. Using FTIR and CD analysis, we found that proteins in embryonic cuticle have high β-sheet secondary structures. Embryonic cuticles displayed high Congo red binding affinity and stained samples showed apple-green birefringence under polarized light, confirming the presence of amyloid-like structures. Amyloid structures have a tendency to propagate and cause amyloidosis. However, feeding of amyloid rich embryonic cuticles to zebrafish did not show any signs of discomfort or morbidity and amyloid deposition. Taken together, the study reveals that amyloid-like structures are present in embryonic cuticle of artemia cyst and their consumption does not induce amyloidosis in zebrafish.
... Historically, the first mention of CJD comes from 1920 [18] and 1921 [19][20][21], when neurologist Hans Gerhard Creutzfeldt and neuropathologist Alfons Maria Jakob described a "nosologically very closely connected if not identical affection" of several patients. Kuru was given attention in the first half of the 20th century as it was described in Papua New Guinea among cannibalistic tribes; the disease is currently considered extinct [22]. The most recent form of TSE, i.e., Variant CJD (vCJD), was first identified in 1996 in the United Kingdom [23,24], which to this day remains the country with the highest number of cases (174) out of a total of 232 worldwide [25]. ...
... Amyloid "kuru" plaques were recorded in 50-75% [74,75] of the examined brains. Currently, kuru disease is considered extinct [22]. ...
Human prion disorders (transmissible spongiform encephalopathies, TSEs) are unique, progressive, and fatal neurodegenerative diseases caused by aggregation of misfolded prion protein in neuronal tissue. Due to the potential transmission, human TSEs are under active surveillance in a majority of countries; in the Czech Republic data are centralized at the National surveillance center (NRL) which has a clinical and a neuropathological subdivision. The aim of our article is to review current knowledge about human TSEs and summarize the experience of active surveillance of human prion diseases in the Czech Republic during the last 20 years. Possible or probable TSEs undergo a mandatory autopsy using a standardized protocol. From 2001 to 2020, 305 cases of sporadic and genetic TSEs including 8 rare cases of Gerstmann–Sträussler–Scheinker syndrome (GSS) were confirmed. Additionally, in the Czech Republic, brain samples from all corneal donors have been tested by the NRL immunology laboratory to increase the safety of corneal transplants since January 2007. All tested 6590 corneal donor brain tissue samples were negative for prion protein deposits. Moreover, the routine use of diagnostic criteria including biomarkers are robust enough, and not even the COVID-19 pandemic has negatively impacted TSEs surveillance in the Czech Republic.
... La découverte de la transmissibilité faite par ce chercheur cumulée aux hypothèses de proximité entre tremblante et kuru permet de faire reconnaître la maladie du kuru comme une encéphalopathie humaine transmissible. D. Carleton Gajdusek a reçu un prix Nobel en 1976 pour ces travaux (Liberski, 2013). La découverte d'une EST humaine ouvre les portes d'un monde encore peu connu. ...
... De plus, les hommes ne consomment que les muscles (contenant peu d'agent pathogène) alors que les femmes et les enfants consomment les bas morceaux riches en prions : cerveau et viscères. Ces traditions expliquent pourquoi les femmes et les enfants ont été plus exposés à la maladie que les hommes (Schwartz, 2001 ;Liberski, 2013). Selon Gadjusek (traduction de l'anglais) : « Il semblerait que le Kuru (…) se soit perpétué uniquement par l'ouverture de la boîte de Pandore qu'est le crâne humain et par la dissémination du virus [comme considéré à l'époque] dans la cavité nasale, les yeux, la peau et peut-être par consommation » (Gadjusek, 1979). ...
... Néanmoins, des patients atteints cliniquement ont présentés des périodes d'incubation extrêmes, de 5 ans et de 60 ans respectivement (Collinge et al., 2008). Le tableau clinique du kuru est décrit (Liberski, 2013) comme évoluant en plusieurs phases. La maladie débute par une phase prodromique qui se caractérise par des douleurs au niveau des articulations ainsi que des maux de tête. ...
... Neurons combination builds the nervous system-brain and spinal cord. Cannibalism is taken as a medium of infection [5]. ...
Kuru is a rare and neurodegenerative disorder-creating disease that effectively and principally affects Neural Synapses and the Central Nervous System. For this reason, brain cells and tissues started to be damaged and deceased. Microbials step out to act against the dangerous deceased cells of the human brain, and spongiform encephalopathy gets created. Thus, the Axon-Dendron of Neuron that contains the human brain gets blocked. In the mid of 19s, Kuru has been revealed as a complicated and a suspected pandemic creating disease. Although most of the diseases are caused by different types of virus, bacteria, fungus etc., Kuru is the first human transmitted disease that has been caused by a complicated and misfolded protein. Human body also bears protein, but that is a normal and not dangerous protein, and it is regarded as Cellular Prion Protein, also known as PrP C. That protein donates itself for the betterment of the human body. But the Scrapie Prion Protein also known as PrPS c is the Prion Protein that causes Kuru Disease and acts against the Brain and Immune System. Actually, Kuru is penetrated through oral intake. The discussion of Alpha-helices and Beta-sheets have been accurately and theoretically revealed from the aspect of Neuroscience in this review paper. Some figures have been added for a better understanding of most relevant and challenging features.
... Neurons combination builds the nervous system-brain and spinal cord. Cannibalism is taken as a medium of infection [5]. ...
... Iatrogenic CJD can be transmitted via medical and surgical procedures such as dura mater grafts and treatment with pituitary-derived growth hormone. Variant CJD emerged after meat products from cattle with bovine spongiform encephalopathy reached the public food chain, and Kuru was initiated by the ritual endocannibalism within indigenous people in Papua New Guinea (6)(7)(8). ...
The purpose of this study was to perform an updated reclassification of all definite prion disease cases with available fresh-frozen samples referred to the Danish Reference Center over the past 40 years, putting a special emphasis on the molecular characterization of novel disease subtypes. Investigation of the Danish prion diseases cohort revealed rare sporadic Creutzfeldt-Jakob disease cases with mixed subtypes and subtypes with previously uncharacterized white matter plaques, a new case of sporadic fatal insomnia, and 3 novel mutations, including 2 large octapeptide repeat insertions, and a point mutation in the prion protein gene. The evaluation of methionine and valine distribution at codon 129 among the prion disease patients in the cohort revealed the increased prevalence of methionine homozygotes compared to the general population. This observation was in line with the prevalence reported in other Caucasian prion disease cohort studies. Reclassification of the old prion diseases cohort revealed unique cases, the molecular characterization of which improves prion diseases classification, diagnostic accuracy, genetic counseling of affected families, and the understanding of disease biology.
... Apart from those, Prion diseases are characterized as the lethal form of neurodegenerative diseases with no clearly defined molecular mechanism and cure. Among the different types of Prion diseases, Kuru is one of the oldest that was discovered in New Guinea [2]. Later, Creudzfelt-Jacob Disease (CJD) was identified for the first time in the UK in late 1990s [3]. ...
... Although one could raise the question of whether all prion diseases are "acquired," the fact that genetic mutations are sufficient to cause prion disease, in combination with the absence of prion disease clusters (except in the case of genetic mutations), argues against this hypothesis. So far, only 3 relatively large-scale instances of transmitted human prion disease have been documented: Kuru among the Fore linguistic group in New Guinea (25), iatrogenic CJD secondary to cadaveric pituitary extract injections (growth hormone and gonadotropins), dura mater grafts, corneal transplants, and contaminated neurosurgical instrumentation (26), and vCJD secondary to ingestion of contaminated beef products (27). Kuru, while of great historical interest, appears (despite extremely long incubation periods in some cases) to have run its course. ...
Prion diseases comprise a group of transmissible degenerative encephalopathies resulting from propagation of a misfolded cellular protein of uncertain function. As is generally the case with rare diseases, lack of institutional experience compromises individual familiarity with the varying, and apparently protean, manifestations of prion diseases, both clinically and pathologically. Coupled with the documented transmissibility of these diseases both within and between species, the Centers for Disease Control and Prevention (CDC) has established the National Prion Disease Pathology Surveillance Center to both aid with diagnosis of prion disease and to survey the United States for evidence of zoonotic transmission. We have assembled this primer with the hope that our accumulated experience will enable the neuropathological community to help the CDC “save lives and protect people.”
