Protective Effect of Val129-PrP against Bovine Spongiform Encephalopathy but not Variant Creutzfeldt-Jakob Disease

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• Alternative Title:
  Emerg Infect Dis
• Personal Author:
  Fernández-Borges, Natalia ; Espinosa, Juan Carlos ; Marín-Moreno, Alba ; Aguilar-Calvo, Patricia ; Asante, Emmanuel A. ; Kitamoto, Tetsuyuki ; Mohri, Shirou ; Andréoletti, Olivier ; Torres, Juan María
• Description:
  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.
• Subjects:
  Bovine Spongiform Encephalopathy BSE Met/Val Methionine Prions Prion Susceptibility PRNP Protective Effect Of Val129-PrP Against Bovine Spongiform Encephalopathy But Not Variant Creutzfeldt-Jakob Disease PrP Research Ruminant Transmissible Spongiform Encephalopathies Transmission Barrier Val129-PrP Valine Variant Creutzfeldt-Jakob Disease VCJD Zoonoses

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• Source:
  Emerg Infect Dis. 23(9):1522-1530.
• Pubmed ID:
  28820136
• Pubmed Central ID:
  PMC5572891
• Document Type:
  Journal Article
• Volume:
  23
• Issue:
  9
• Collection(s):
  Emerging Infectious Diseases
• Main Document Checksum:
  urn:sha256:fe4388fe76967141c7bee9b3fd32eab9fd5ac89cff9d5290d4f9d20a7c58dd1b
• Download URL:
  https://stacks.cdc.gov/view/cdc/47957/cdc_47957_DS1.pdf
• File Type:
  [PDF - 1.14 MB ]