ArticleProceedings of the National Academy of Sciences of the United States of America2008
Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 44 papers.
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44 citing papers in PubMed, 90 citations in OpenAlex.
- Strain-specific propagation of variant Creutzfeldt-Jakob disease prions in humanized neural cells.Proceedings of the National Academy of Sciences of the United States of America · 2026Article
- Isolation of a novel human prion strain from a PRNP codon 129 heterozygous vCJD patient.PLoS pathogens · 2025Article
- Review
- Sporadic Creutzfeldt-Jakob disease infected human cerebral organoids retain the original human brain subtype features following transmission to humanized transgenic mice.Acta neuropathologica communications · 2023Article
- Transmission characteristics of heterozygous cases of Creutzfeldt-Jakob disease with variable abnormal prion protein allotypes.Acta neuropathologica communications · 2020Article
- Spontaneous generation of prions and transmissible PrP amyloid in a humanised transgenic mouse model of A117V GSS.PLoS biology · 2020Article
- Protein aggregate formation permits millennium-old brain preservation.Journal of the Royal Society, Interface · 2020Article
- Understanding Prion Strains: Evidence from Studies of the Disease Forms Affecting Humans.Viruses · 2019Review
- Kuru, the First Human Prion Disease.Viruses · 2019Review
- Recent advances in the histo-molecular pathology of human prion disease.Brain pathology (Zurich, Switzerland) · 2019Review
- A new era for understanding amyloid structures and disease.Nature reviews. Molecular cell biology · 2018Review
- Experimental sheep BSE prions generate the vCJD phenotype when serially passaged in transgenic mice expressing human prion protein.Journal of the neurological sciences · 2018Article
- Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein.Acta neuropathologica communications · 2018Article
- Rapid Testing for Creutzfeldt-Jakob Disease in Donors of Cornea.Transplantation · 2017Article
- Prion disease: experimental models and reality.Acta neuropathologica · 2017Review
- Sporadic and Infectious Human Prion Diseases.Cold Spring Harbor perspectives in medicine · 2017Review
- Cultural factors that affected the spatial and temporal epidemiology of kuru.Royal Society open science · 2017Article
- Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years.Brain : a journal of neurology · 2015Article
- Transmission Properties of Human PrP 102L Prions Challenge the Relevance of Mouse Models of GSS.PLoS pathogens · 2015Article
- A naturally occurring variant of the human prion protein completely prevents prion disease.Nature · 2015Article
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Authors and funding
12 authors at 3 institutions in 1 country.
Funding
Abstract
Kuru provides our principal experience of an epidemic human prion disease and primarily affected the Fore linguistic group of the Eastern Highlands of Papua New Guinea. Kuru was transmitted by the practice of consuming dead relatives as a mark of respect and mourning (transumption). To date, detailed information of the prion strain type propagated in kuru has been lacking. Here, we directly compare the transmission properties of kuru prions with sporadic, iatrogenic, and variant Creutzfeldt-Jakob disease (CJD) prions in Prnp-null transgenic mice expressing human prion protein and in wild-type mice. Molecular and neuropathological data from these transmissions show that kuru prions are distinct from variant CJD and have transmission properties equivalent to those of classical (sporadic) CJD prions. These findings are consistent with the hypothesis that kuru originated from chance consumption of an individual with sporadic CJD.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.