vCJD prion acquires altered virulence through trans-species infection

Masahiro Asano, Shirou Mohri, James W. Ironside, Mamoru Ito, Norikazu Tamaoki, Tetsuyuki Kitamoto

Research output: Contribution to journalArticlepeer-review

44 Citations (Scopus)


Variant Creutzfeldt-Jakob disease (vCJD) appears to be caused by infection with the bovine spongiform encephalopathy (BSE) agent. To date, all patients with vCJD are homozygous for methionine at codon 129 of the PrP gene. To investigate the relationship between polymorphism at codon 129 and susceptibility to BSE or vCJD prions, we performed splenic follicular dendritic cell assay with humanized knock-in mice through peripheral infection. All humanized knock-in mice showed little or no susceptibility to BSE prions. Only the subset of humanized knock-in mice with codon 129 Met/Met genotype showed weak susceptibility by Western blotting. Surprisingly, we succeeded in the transmission of vCJD prions to humanized knock-in mice not only with codon 129 Met/Met but also with codon 129 Met/Val. Humanized knock-in mice with codon 129 Val/Val were not susceptible. The results suggest that human heterozygotes at codon 129 are also at risk for secondary infection with vCJD.

Original languageEnglish
Pages (from-to)293-299
Number of pages7
JournalBiochemical and biophysical research communications
Issue number1
Publication statusPublished - 2006 Mar 31


  • BSE
  • Bovinized mouse
  • Follicular dendritic cell
  • Humanized mouse
  • Polymorphism
  • PrP
  • Prion
  • Traceback
  • Virulence
  • vCJD

ASJC Scopus subject areas

  • Biophysics
  • Biochemistry
  • Molecular Biology
  • Cell Biology


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