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

Yoshiko Munesue, Taishi Shimazaki, Zechen Qi, Norikazu Isoda, Hirofumi Sawa, Keisuke Aoshima, Takashi Kimura, Shirou Mohri, Tetsuyuki Kitamoto, Atsushi Kobayashi

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)43-47
Number of pages5
JournalNeuroscience Letters
Volume668
DOIs
Publication statusPublished - 2018 Mar 6

Keywords

  • Creutzfeldt-Jakob disease
  • Follicular dendritic cell
  • Knock-in mouse
  • Prion
  • Transmissible spongiform encephalopathy
  • Transmission property

ASJC Scopus subject areas

  • Neuroscience(all)

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