Development of a quick bioassay for the evaluation of transmission properties of acquired prion diseases

被引:5
作者
Munesue, Yoshiko [1 ]
Shimazaki, Taishi [1 ]
Qi, Zechen [1 ]
Isoda, Norikazu [2 ,3 ]
Sawa, Hirofumi [2 ,4 ]
Aoshima, Keisuke [1 ]
Kimura, Takashi [1 ]
Mohri, Shirou [5 ]
Kitamoto, Tetsuyuki [5 ]
Kobayashi, Atsushi [1 ]
机构
[1] Hokkaido Univ, Fac Vet Med, Lab Comparat Pathol, Kita Ku, Kita 18 Nishi 9, Sapporo, Hokkaido 0600818, Japan
[2] Hokkaido Univ, Global Inst Collaborat Res & Educ GI CoRE, Global Stn Zoonosis Control, Sapporo, Hokkaido, Japan
[3] Hokkaido Univ, Res Ctr Zoonosis Control, Unit Risk Anal & Management, Kita Ku, Kita 20 Nishi 10, Sapporo, Hokkaido 0010020, Japan
[4] Hokkaido Univ, Res Ctr Zoonosis Control, Div Mol Pathobiol, Kita Ku, Kita 20 Nishi 10, Sapporo, Hokkaido 0010020, Japan
[5] Tohoku Univ, Grad Sch Med, Dept Neurol Sci, Aoba Ku, 2-1 Seiryo Machi, Sendai, Miyagi 9808575, Japan
关键词
Transmissible spongiform encephalopathy; Prion; Creutzfeldt-Jakob disease; Follicular dendritic cell; Transmission property; Knock-in mouse; CREUTZFELDT-JAKOB-DISEASE; CYCLIC AMPLIFICATION; ABNORMAL ISOFORM; PROTEIN; MICE; QUINACRINE; INFECTION; SEQUENCE; PLAQUES; CELL;
D O I
10.1016/j.neulet.2018.01.014
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
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.
引用
收藏
页码:43 / 47
页数:5
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