Kuru

A prion disease found exclusively among the Fore linguistic group natives of the highlands of NEW GUINEA. The illness is primarily restricted to adult females and children of both sexes. It is marked by the subacute onset of tremor and ataxia followed by motor weakness and incontinence. Death occurs within 3-6 months of disease onset. The condition is associated with ritual cannibalism, and has become rare since this practice has been discontinued. Pathologic features include a noninflammatory loss of neurons that is most prominent in the cerebellum, glial proliferation, and amyloid plaques. (From Adams et al., Principles of Neurology, 6th ed, p773)

Also Known As:

Networked: 220 relevant articles (2 outcomes, 11 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Scrapie
2.	Acquired CJD
3.	Sporadic Creutzfeldt-Jakob Disease
4.	Bovine Spongiform Encephalopathy (Mad Cow Disease)
5.	Creutzfeldt-Jakob Syndrome (Creutzfeldt-Jakob Disease)

Experts

1.	Collinge, John: 12 articles (12/2019 - 06/2006)
2.	Mead, Simon: 8 articles (12/2019 - 06/2006)
3.	Brandner, Sebastian: 7 articles (11/2015 - 03/2008)
4.	Kitamoto, Tetsuyuki: 6 articles (10/2018 - 08/2006)
5.	Whitfield, Jerome: 6 articles (06/2015 - 06/2006)
6.	Alpers, Michael P: 6 articles (11/2009 - 06/2006)
7.	Parchi, Piero: 5 articles (11/2017 - 10/2010)
8.	Wadsworth, Jonathan D F: 5 articles (11/2015 - 03/2008)
9.	Liberski, Paweł P: 4 articles (01/2019 - 01/2004)
10.	Sikorska, Beata: 4 articles (01/2019 - 01/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Kuru:

1.	PrionsIBA 06/25/2015 - "Notably, mice expressing only PrP V127 were completely resistant to all prion strains, demonstrating a different molecular mechanism to M129V, which provides its relative protection against classical CJD and kuru in the heterozygous state. " 01/01/2023 - "Interestingly, cells expressing BVPrP containing the G127V prion gene variant, identified in individuals resistant to kuru, were unable to become infected with prions. " 09/01/2019 - "The Cerebellar form originated by the VV2 prion occurs in 15% of cases, the form with Kuru plates, associated with the prion MV2 occurs in 5%, and the Vacuolar, related to the MM2 prion occurs in 4%. " 11/01/2015 - "All but one showed synaptic prion deposition with numerous kuru type plaques in the basal ganglia, anterior frontal and parietal cortex, thalamus, basal ganglia and cerebellum. " 11/26/2013 - "The MSA prion represents a unique human pathogen that is lethal upon transmission to Tg mice and as such, is reminiscent of the prion causing kuru, which was transmitted to chimpanzees nearly 5 decades ago. "
2.	Amyloid (Amyloid Fibrils)IBA 01/01/1998 - "Some PrP deposits are also of the amyloid variety (kuru type, multicentric or florid plaques), but immunohistochemistry, far more sensitive than conventional studies, revealed a number of other lesions (perivacuolar, neuronal, "synaptic" deposits...). " 01/01/2020 - "The second variant, denoted sCJDMV2K, features prominent deposition of PrPD amyloid forming kuru type (K) plaques. " 01/01/2012 - "Neuropathologically, kuru is characterized by the presence of amyloid "kuru" plaques." 01/01/2004 - "Unicentric "kuru" plaques consisted of stellate arrangements (stars or cores) of amyloid bundles emanating from a dense interwoven centre. " 09/01/2001 - "Uniform topographical central nervous system histopathology includes spongiform change and neuronal loss, with amyloid (kuru) plaques in approximately 75% of cases."
3.	Prion ProteinsIBA 03/07/2019 - "Prion protein immunohistochemistry revealed widespread diffuse microvacuolar staining without kuru-type plaques. " 11/01/2015 - "The patient with the shortest clinical duration had an atypical synaptic deposition of abnormal prion protein and no kuru plaques. " 02/01/2012 - "Kuru imposed a strong balancing selection on the Fore population, with individuals homozygous for the 129 Met allele of the gene (PRNP) encoding for prion protein (PrP) being the most susceptible. " 11/19/2009 - "A novel protective prion protein variant that colocalizes with kuru exposure." 08/11/2009 - "We transmitted primate kuru, a TSE once epidemic in New Guinea, to mice expressing normal and approximately 8-fold higher levels of murine prion protein (PrP). "
4.	AntibodiesIBA 12/01/1989 - "Antibodies on the Western blot membrane from murine spleen PrPCJD fractions stained the kuru plaques in the CJD-infected mouse brain. " 03/01/1978 - "A serological survey is reported for antibodies to Coxsackie, ECHO and polioviruses on sera collected in the kuru region of Papua New Guinea during earliest European contacts with kuru-affected and control populations through the period of highest kuru incidence. " 01/01/1999 - "Kuru transmitted by cannibalism involved mainly the cerebellum with cerebellar tracts degeneration and presence of amyloid plaques called "kuru plaques" labeled by anti PrP antibodies. " 03/01/1990 - "Antibodies prepared against mouse and hamster PrP27-30 reacted with amyloid plaques in scrapie-infected mouse and kuru- and CJD-infected human brain sections but not with amyloid plaques in the brain of a patient with Alzheimer's disease." 01/01/2008 - "Distinct immunohistochemical localization in Kuru plaques using novel anti-prion protein antibodies."
5.	Methionine (L-Methionine)FDA Link 01/15/2001 - "Increased susceptibility to Kuru of carriers of the PRNP 129 methionine/methionine genotype." 06/01/2004 - "Recent studies indicate that individuals homozygous for Methionine at a polymorphic position 129 of the prion protein were preferentially affected during the kuru epidemic. " 11/19/2009 - "We now report a novel PRNP variant--G127V--that was found exclusively in people who lived in the region in which kuru was prevalent and that was present in half of the otherwise susceptible women from the region of highest exposure who were homozygous for methionine at PRNP codon 129. " 01/15/2001 - "Analysis of the PRNP 129 methionine (M)/valine (V) polymorphism in 80 patients and 95 unaffected controls demonstrated that the kuru epidemic preferentially affected individuals with the M/M genotype. " 06/01/2016 - "In this model, the inoculation of the sporadic CJD strain V2 into animals homozygous for methionine at polymorphic codon 129 (129 M/M) of the PRNP gene produced quite distinctive neuropathological and biochemical features, that is, widespread kuru plaques and intermediate type abnormal PrP (PrP(Sc) ). "
6.	Proteins (Proteins, Gene)FDA Link 01/01/1992 - "Kuru plaques are composed of this protein. " 10/01/1988 - "In addition, the anti-beta protein antiserum immunolabeled kuru plaque-like compact plaques in some cases of CJD. " 11/01/1972 - "3. Distribution of the inherited serum group-specific protein (Ge) phenotypes in New Guineans: an association of kuru and the Gc Ab phenotype." 01/03/2004 - "Notwithstanding the generally low incidence of TSE and their limited infectiousness, major epidemics such as bovine spongiform encephalopathy and kuru arise in situations where intraspecies recycling of the abnormal protein is sustained. " 01/01/2004 - "Kuru research has impacted the concepts of nucleation-polymerization "protein cancers", and "conformational disorders". "
7.	Codon (Codons)IBA 08/06/2013 - "To elucidate the epidemiological mechanism of human resistance, we estimated the incubation period and transmission rate of kuru for codon 129 heterozygotes and homozygotes using kuru incidence data and human genotype frequency data from 1957 to 2004. " 11/27/2008 - "Elderly survivors of the kuru epidemic, who had multiple exposures at mortuary feasts, are predominantly PRNP codon 129 heterozygotes and this group show marked Hardy-Weinberg disequilibrium. " 11/27/2008 - "In these patients, the mean clinical duration of 17 months was longer than the overall average in kuru but similar to that previously reported for the same age group, and this may relate to the effects of both patient age and PRNP codon 129 genotype. " 06/24/2006 - "PRNP analysis showed that most patients with kuru were heterozygous at polymorphic codon 129, a genotype associated with extended incubation periods and resistance to prion disease. " 10/27/1998 - "If nvCJD behaves like kuru, future cases (with longer incubation periods) may begin to occur in older individuals with heterozygous codon 129 genotypes and signal a maturing evolution of the nvCJD "epidemic." The clinical phenotype of such cases should be similar to that of homozygous cases, but may have less (or at least less readily identified) amyloid plaque formation."
8.	Protein Isoforms (Isoforms)IBA 03/01/1995 - "Its post-translational modification produces an accumulation of abnormal isoform PrPsc found in brains of transmissible neurodegenerative disorders in animals (scrapie and bovine spongiform encephalopathy) and humans (Kuru, Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker syndrome). " 09/01/2013 - "The prionopathies [Kuru, Creutzfeldt-Jakob disease (CJD) and its variants, Gerstmann-Sträussler-Scheinker (GSS) syndrome and fatal familial insomnia (FFI)] result from accumulation of abnormal isoforms of the prion protein in the brains of normal animals on both neuronal and non-neuronal cells. " 10/15/1999 - "Creutzfeldt-Jakob disease, kuru, scrapie and bovine spongiform encephalopathy are diseases of the mammalian central nervous system that involve the conversion of a cellular protein into an insoluble extracellular isoform. " 01/01/2018 - "While loss of function of PrP does not elicit apparent phenotypes, generation of misfolded forms of the protein or its aberrant metabolic isoforms has been implicated in a number of neurodegenerative disorders such as scrapie, kuru, Creutzfeldt-Jakob disease, fatal familial insomnia, Gerstmann-Sträussler-Scheinker and bovine spongiform encephalopathy. " 01/01/2016 - "p-dCJD shows distinctive phenotypic features, namely numerous kuru plaques and an abnormal isoform of prion protein (PrP(Sc)) intermediate in size between types 1 and 2. Transmission studies have shown that the unusual phenotypic features of p-dCJD are linked to the V2 prion strain that is associated with sCJD subtypes VV2 or -MV2. "
9.	Valine (L-Valine)FDA Link 01/15/2001 - "Analysis of the PRNP 129 methionine (M)/valine (V) polymorphism in 80 patients and 95 unaffected controls demonstrated that the kuru epidemic preferentially affected individuals with the M/M genotype. " 12/01/1996 - "Also they did not have a valine polymorphism at PrP codon 129 which is frequently observed in the patients with iatrogenic CJD and kuru. " 11/27/2008 - "Genotype analysis of the polymorphism on codon 129 reveals (in conjunction with updated information from more kuru cases) that all three genotypes (VV, MV and MM (where M is methionine and V is valine)) are detected in kuru with some preference for MM homozygosity. " 08/01/1999 - "Seventy percent of subjects showed the classic CJD phenotype, PrP(Sc) type 1, and at least one methionine allele at codon 129; 25% of cases displayed the ataxic and kuru-plaque variants, associated to PrP(Sc) type 2, and valine homozygosity or heterozygosity at codon 129, respectively. " 06/25/2015 - "Autopsy examination revealed a neuropathological phenotype reminiscent of both sCJDMV2-K (a sporadic CJD subtype in subjects methionine/valine heterozygous at codon 129 with PrP(Sc) type 2 and the presence of kuru plaques) and variant CJD (vCJD). "
10.	ParaffinIBA 02/01/2009 - "Immunohistochemistry was performed on paraffin sections from one case of vCJD, two cases of GSS, one case of kuru and two cases of sCJD. " 01/01/2019 - "We provide descriptions of electron microscopy and confocal microscopy of kuru amyloid plaques retrieved from a paraffin-embedded block of an old kuru case, named Kupenota. " 03/01/2003 - "We examined paraffin-embedded brain sections of sporadic MV2 Creutzfeldt-Jakob disease (sCJD) with Kuru plaques, sporadic VV2 CJD with plaque-like PrP(sc) (the abnornal form of prion protein) deposits, variant CJD (vCJD) with florid plaques, Gerstmann-Straüssler-Scheinker (GSS) with multicentric plaques and of Alzheimer's disease (AD) with senile plaques. " 09/03/2003 - "The study aimed to evaluate the fluorescent molecular-imaging probe 2-(1-[6-[(2-fluoroethyl)(methyl)amino]-2-naphthyl]ethylidene)malononitrile (FDDNP) for its ability to selectively and reproducibly label prion plaques in fixed, paraffin-embedded cerebellar sections from patients of confirmed Gerstmann-Sträussler-Scheinker disease, sporadic Creutzfeldt-Jacob disease (CJD) with kuru plaques, and variant CJD (vCJD). " 12/01/1991 - "Formic acid pretreatment on formalin fixed, paraffin-embedded thin sections enhanced immunostaining of PrP in both congophilic and non-congophilic kuru plaques which were absent in sporadic CJD patients with short clinical courses. "

Therapies and Procedures

1.	Pancreaticoduodenectomy 03/01/2020 - "The first report of pancreatoduodenectomy was the abstract of Japan Surgical Society in 1946 by Kuru, followed by a publication by Yoshioka (Geka, 1950). "
2.	Therapeutics 09/01/1993 - "The clinical features of all such reported cases resemble more closely those of sporadic CJD, in contrast with kuru and the cases of CJD which have arisen after therapy with human pituitary-derived growth hormone. "
3.	Intramuscular Injections 04/01/1975 - "Following intramuscular injection of Kuru and Jakob-Creutzfeldt brain material into chimpanzees, circulating lymphocytes became sensitized to scrapie mouse brain (and spleen) to a greater degree than to normal tissue. "
4.	Proctectomy 01/01/1988 - "Kuru reported the superiority of abdominoperineal resection (APR), an orient of surgery had changed to APR from ASE with time. "