Munesue Yoshiko, Shimazaki Taishi, Qi Zechen, Isoda Norikazu, Sawa Hirofumi, Aoshima Keisuke, Kimura Takashi, Mohri Shirou, Kitamoto Tetsuyuki, Kobayashi Atsushi
Laboratory of Comparative Pathology, Faculty of Veterinary Medicine, Hokkaido University, Kita 18 Nishi 9, Kita-ku, Sapporo, 060-0818, Japan.
Global Station for Zoonosis Control, Global Institute for Collaborative Research and Education (GI-CoRE), Hokkaido University, Japan; Unit of Risk Analysis and Management, Research Center for Zoonosis Control, Hokkaido University, Kita 20 Nishi 10, Kita-ku, Sapporo, 001-0020, Japan.
Neurosci Lett. 2018 Mar 6;668:43-47. doi: 10.1016/j.neulet.2018.01.014. Epub 2018 Jan 9.
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 the PRNP gene, 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 same PRNP codon 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.
对于一组获得性克雅氏病（CJD）进行鉴别诊断而言，评估传播特性至关重要。这组疾病在朊蛋白基因（PRNP）多态性密码子129处为甲硫氨酸纯合子，具有中间型异常朊蛋白（PrP）以及库鲁氏斑，被称为获得性CJD-MMiK。本研究旨在开发一种快速评估获得性CJD-MMiK传播特性的系统。在用一名获得性CJD-MMiK患者的脑匀浆腹腔接种的PrP人源化小鼠中，接种后75天在脾脏的滤泡树突状细胞中观察到了异常PrP的积累。获得性CJD-MMiK的传播特性与具有相同PRNP密码子129基因型的散发性CJD的传播特性截然不同。此外,即使在接种后14天，也能够检测到获得性CJD-MMiK的特征性传播特性。这些发现表明，使用脾脏滤泡树突状细胞的生物测定法（称为FDC测定法）可以成为一种简便、省时且有用的方法，用于区分获得性CJD-MMiK和散发性CJD。
