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

Research output: Contribution to journalArticlepeer-review


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 (PrP(Sc)) of prion protein (PrP) accumulates in follicular dendritic cells within lymphoid organs. Human prions, however, did not cause an accumulation of PrP(Sc) in the wild type mice. Here, we report that knock-in mouse expressing humanized chimeric PrP demonstrated PrP(Sc) accumulations in follicular dendritic cells following human prion infections, including variant Creutzfeldt-Jakob disease. The accumulated PrP(Sc) 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.

Original languageEnglish
Pages (from-to)280-6
Number of pages7
JournalBiochemical and Biophysical Research Communications
Issue number2
Publication statusPublished - 7 Jun 2002


  • Animals
  • Biological Assay
  • Brain
  • Creutzfeldt-Jakob Syndrome
  • Dendritic Cells, Follicular
  • Disease Models, Animal
  • Genetic Predisposition to Disease
  • Humans
  • Immunohistochemistry
  • Mice
  • Mice, Transgenic
  • PrPSc Proteins
  • Prion Diseases
  • Prions
  • Recombinant Fusion Proteins
  • Spleen


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