BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing

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Updated: Sep 28, 2026

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans
12:57

Investigating the Spreading and Toxicity of Prion-like Proteins Using the Metazoan Model Organism C. elegans

Published on: January 8, 2015

Emmanuel A Asante1, Jacqueline M Linehan, Melanie Desbruslais

  • 1MRC Prion Unit, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK.

The EMBO Journal
|November 29, 2002
PubMed

Insights

Transgenic mice exposed to bovine spongiform encephalopathy (BSE) or variant Creutzfeldt-Jakob disease (vCJD) prions can develop vCJD-like disease. BSE exposure may also cause a sporadic CJD-like phenotype, suggesting multiple BSE prion strains can infect humans.

Area of Science:

  • Neuroscience
  • Prion Diseases
  • Molecular Biology

Background:

  • Variant Creutzfeldt-Jakob disease (vCJD) has been exclusively observed in individuals homozygous for methionine at the PRNP codon 129.
  • Bovine spongiform encephalopathy (BSE) and vCJD are believed to be caused by the same prion strain.

Purpose of the Study:

  • To investigate the potential for BSE prions to cause distinct prion disease phenotypes in a relevant animal model.
  • To explore the possibility of multiple BSE-derived prion strains infecting humans.

Main Methods:

  • Transgenic mice expressing human PrP methionine at codon 129 were inoculated with BSE or vCJD prions.
  • Neuropathological and molecular analyses were performed to characterize the resulting prion diseases.

Main Results:

  • Inoculation with BSE or vCJD prions induced a vCJD-like neuropathological and molecular phenotype in the transgenic mice.
  • Surprisingly, BSE exposure also resulted in a distinct molecular phenotype resembling sporadic CJD with PrP(Sc) type 2.
  • These findings indicate that BSE can generate at least two distinct prion strains.

Conclusions:

  • BSE prion exposure in humans may lead to phenotypes beyond vCJD, including those resembling sporadic CJD.
  • The study suggests that some cases of sporadic CJD might be linked to prior BSE exposure.
  • This highlights the complex nature of prion strain diversity and zoonotic potential.

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