1. Development of a quick bioassay for the evaluation of transmission properties of acquired prion diseases
- Author
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Hirofumi Sawa, Yoshiko Munesue, Atsushi Kobayashi, Keisuke Aoshima, Takashi Kimura, Zechen Qi, Norikazu Isoda, Taishi Shimazaki, Shirou Mohri, and Tetsuyuki Kitamoto
- Subjects
0301 basic medicine ,animal diseases ,Mice, Transgenic ,Spleen ,Biology ,Creutzfeldt-Jakob Syndrome ,PRNP ,Mice ,03 medical and health sciences ,chemistry.chemical_compound ,mental disorders ,Genotype ,medicine ,Animals ,Humans ,Methionine ,Transmissible spongiform encephalopathy ,Follicular dendritic cells ,General Neuroscience ,medicine.disease ,Virology ,nervous system diseases ,Disease Models, Animal ,030104 developmental biology ,medicine.anatomical_structure ,chemistry ,Kuru ,Biological Assay ,Dendritic Cells, Follicular - Abstract
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.
- Published
- 2018
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