Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice

Wadsworth JDF; Joiner S; Linehan JMDesbruslais MFox KCooper SCronier SAsante EAMead SBrandner SHill AFCollinge J

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Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice

机译：Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice

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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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《Proceedings of the National Academy of Sciences of the United States of America》 |2008年第10期|3885-3890|共6页
作者
Wadsworth JDF; Joiner S; Linehan JMDesbruslais MFox KCooper SCronier SAsante EAMead SBrandner SHill AFCollinge J;
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作者单位

UCL, Neurol Inst, Natl Hosp Neurol & Neurosurg, Prion Unit,Med Res Council, London WC1N 3BG, England.;

UCL, Neurol Inst, Natl Hosp Neurol & Neurosurg, Dept Neurodegenerat Dis, London WC1N 3BG, England.;

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收录信息美国《科学引文索引》(SCI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
原文格式 PDF
正文语种英语
中图分类自然科学总论;
关键词
variant CJD; transmissible spongiform encephalopathy; iatrogenic CJD;

Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice

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