Follicular dendritic cell of the knock-in mouse provides a new bioassay for human prions

Tetsuyuki Kitamoto, Shirou Mohri, James W. Ironside, Ichiro Miyoshi, Tomoyuki Tanaka, Noritoshi Kitamoto, Shigeyoshi Itohara, Noriyuki Kasai, Motoya Katsuki, Jun Higuchi, Tamaki Muramoto, Ryong Woon Shin

研究成果: Article査読

42 被引用数 (Scopus)


Infectious prion diseases initiate infection within lymphoid organs where prion infectivity accumulates during the early stages of peripheral infection. In a mouse-adapted prion infection, an abnormal isoform (PrPSc) of prion protein (PrP) accumulates in follicular dendritic cells within lymphoid organs. Human prions, however, did not cause an accumulation of PrPSc in the wild type mice. Here, we report that knock-in mouse expressing humanized chimeric PrP demonstrated PrPSc accumulations in follicular dendritic cells following human prion infections, including variant Creutzfeldt-Jakob disease. The accumulated PrPSc consisted of recombinant PrP, but not of the inoculated human PrP. These accumulations were detectable in the spleens of all mice examined 30 days post-inoculation. Infectivity of the spleen was also evident. Conversion of humanized PrP in the spleen provides a rapid and sensitive bioassay method to uncover the infectivity of human prions. This model should facilitate the prevention of infectious prion diseases.

ジャーナルBiochemical and biophysical research communications
出版ステータスPublished - 2002

ASJC Scopus subject areas

  • 生物理学
  • 生化学
  • 分子生物学
  • 細胞生物学


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