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Manuelidis, L, Chakrabarty, T, Miyazawa, K, Nduom, NA and Emmerling, K (2009) The kuru infectious agent is a unique geographic isolate distinct from Creutzfeldt-Jakob disease and scrapie agents. Proc. Natl. Acad. Sci. U.S.A. 106:13529-34


Human sporadic Creutzfeldt-Jakob disease (sCJD), endemic sheep scrapie, and epidemic bovine spongiform encephalopathy (BSE) are caused by a related group of infectious agents. The new U.K. BSE agent spread to many species, including humans, and clarifying the origin, specificity, virulence, and diversity of these agents is critical, particularly because infected humans do not develop disease for many years. As with viruses, transmissible spongiform encephalopathy (TSE) agents can adapt to new species and become more virulent yet maintain fundamentally unique and stable identities. To make agent differences manifest, one must keep the host genotype constant. Many TSE agents have revealed their independent identities in normal mice. 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). High levels of murine PrP did not prevent infection but instead shortened incubation time, as would be expected for a viral receptor. Sporadic CJD and BSE agents and representative scrapie agents were clearly different from kuru in incubation time, brain neuropathology, and lymphoreticular involvement. Many TSE agents can infect monotypic cultured GT1 cells, and unlike sporadic CJD isolates, kuru rapidly and stably infected these cells. The geographic independence of the kuru agent provides additional reasons to explore causal environmental pathogens in these infectious neurodegenerative diseases.


PubMed PMC2715327 Online version:10.1073/pnas.0905825106


Animals; Brain/pathology; Cattle; Cells, Cultured; Creutzfeldt-Jakob Syndrome/pathology; Creutzfeldt-Jakob Syndrome/transmission; Geography; Humans; Kuru/pathology; Kuru/transmission; Mice; Neurons/pathology; Prions/isolation & purification; Prions/metabolism; Scrapie/pathology; Sheep; Time Factors; Tissue Culture Techniques



Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status


GO:0043524 : negative regulation of apoptotic process



Fig. 2

CACAO 7825

See also


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