Protective effect of Val129-PrP against bovine spongiform encephalopathy but not variant Creutzfeldt-Jakob disease

Natalia Fernández-Borges, Juan Carlos Espinosa, Alba Marín-Moreno, Patricia Aguilar-Calvo, Emmanuel A. Asante, Tetsuyuki Kitamoto, Shirou Mohri, Olivier Andréoletti, Juan María Torres Trillo

Research output: Contribution to journalArticlepeer-review

16 Citations (Scopus)

Abstract

Bovine spongiform encephalopathy (BSE) is the only known zoonotic prion that causes variant Creutzfeldt-Jakob disease (vCJD) in humans. The major risk determinant for this disease is the polymorphic codon 129 of the human prion protein (Hu-PrP), where either methionine (Met129) or valine (Val129) can be encoded. To date, all clinical and neuropathologically confirmed vCJD cases have been Met129 homozygous, with the exception of 1 recently reported Met/Val heterozygous case. Here, we found that transgenic mice homozygous for Val129 Hu-PrP show severely restricted propagation of the BSE prion strain, but this constraint can be partially overcome by adaptation of the BSE agent to the Met129 Hu-PrP. In addition, the transmission of vCJD to transgenic mice homozygous for Val129 Hu-PrP resulted in a prion with distinct strain features. These observations may indicate increased risk for vCJD secondary transmission in Val129 Hu-PrP–positive humans with the emergence of new strain features.

Original languageEnglish
Pages (from-to)1522-1530
Number of pages9
JournalEmerging Infectious Diseases
Volume23
Issue number9
DOIs
Publication statusPublished - 2017 Sep

ASJC Scopus subject areas

  • Epidemiology
  • Microbiology (medical)
  • Infectious Diseases

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