Kuru Prions and Sporadic Creutzfeldt-Jakob Disease Prions Have Equivalent Transmission Properties in Transgenic and Wild-Type Mice

• Published in: Proceedings of the National Academy of Sciences - PNAS Vol. 105; no. 10; pp. 3885 - 3890
• Main Authors: Wadsworth, Jonathan D. F., Joiner, Susan, Linehan, Jacqueline M., Desbruslais, Melanie, Fox, Katie, Cooper, Sharon, Cronier, Sabrina, Asante, Emmanuel A., Mead, Simon, Brandner, Sebastian, Hill, Andrew F., Collinge, John
• Format: Journal Article
• Language: English
• Published: United States National Academy of Sciences 11-03-2008; National Acad Sciences
• Subjects: Animals; Biological Sciences; Brain - pathology; Codons; Creutzfeldt Jakob syndrome; Creutzfeldt-Jakob disease; Creutzfeldt-Jakob Syndrome - pathology; Creutzfeldt-Jakob Syndrome - transmission; Disease transmission; Humans; Immunoblotting; Infections; Kuru; Kuru - pathology; Kuru - transmission; Mice; Mice, Transgenic; Pathology; Phenotype; Prion diseases; Prions; Prions - metabolism; Proteins; PrPSc Proteins - metabolism; Rodents; Transgenic animals; Papua New Guinea
• ISSN: 0027-8424; 1091-6490
• DOI: 10.1073/pnas.0800190105

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.