Development of a quick bioassay for the evaluation of transmission properties of acquired prion diseases.
Development of a quick bioassay for the evaluation of transmission properties of acquired prion diseases.
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开发一种快速生物测定法，用于评估获得性朊病毒疾病的传播特性。
DOI：
10.1016/j.neulet.2018.01.014
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发表时间：
2018
影响因子：
2.5
通讯作者：
Kobayashi A
中科院分区：
文献类型：
--
作者：
Munesue Y;Shimazaki T;Qi Z;Isoda N;Sawa H;Aoshima K;Kimura T;Mohri S;Kitamoto T;Kobayashi A
对于PRNP基因129位蛋氨酸纯合的获得性克雅病(CJD)亚群、一种中间型异常蛋白(PrP)和库鲁斑(称为获得性CJD-MMiK)的传播特性的评估是重要的鉴别诊断。本研究旨在开发一种快速评价CJD-MMiK的传输性能的系统。将一位获得性CJD-MMiK患者的脑组织匀浆经腹膜接种PrP人源化小鼠，在接种后75 天，可在脾滤泡树突状细胞中观察到异常PrP的聚集。获得性CJD-MMiK的传播特性与相同PRNP密码子129基因的散发性CJD有很大不同。此外，即使在接种后14 天，也可以检测到获得的CJD-MMiK的特征传播特性。这些结果表明，利用脾滤泡树突状细胞的生物测定，即FDC测定，是鉴别获得性CJD-MMiK和散发性CJD的一种简便、省时和有用的方法。
Evaluation of transmission properties is important for the differential diagnosis of a subgroup of acquired Creutzfeldt-Jakob disease (CJD) with methionine homozygosity at polymorphic codon 129 of thePRNPgene, an intermediate type abnormal prion protein (PrP), and kuru plaques, denoted as acquired CJD-MMiK. The present study aimed to develop a quick evaluation system of the transmission properties of acquired CJD-MMiK. In the PrP-humanized mice intraperitoneally inoculated with brain homogenates from an acquired CJD-MMiK patient, accumulation of abnormal PrP was observed in follicular dendritic cells of the spleen at 75 days post-inoculation. The transmission properties of acquired CJD-MMiK were quite different from those of sporadic CJD with the samePRNPcodon 129 genotype. Moreover, even at 14 days post-inoculation, the characteristic transmission properties of acquired CJD-MMiK could be detected. These findings suggest that the bioassay using follicular dendritic cells of the spleen, named as a FDC assay, can be an easy, time-saving, and useful method to distinguish acquired CJD-MMiK from sporadic CJD.