Classical Bovine Spongiform Encephalopathy by Transmission of H-Type Prion in Homologous Prion Protein Context

Details

• Alternative Title:
  Emerg Infect Dis
• Personal Author:
  Torres, Juan-María ; Andréoletti, Olivier ; Lacroux, Caroline ; Prieto, Irene ; Lorenzo, Patricia ; Larska, Magdalena ; Baron, Thierry ; Espinosa, Juan-Carlos
• Description:
  Bovine spongiform encephalopathy (BSE) and BSE-related disorders have been associated with a single major prion strain. Recently, 2 atypical, presumably sporadic forms of BSE have been associated with 2 distinct prion strains that are characterized mainly by distinct Western blot profiles of abnormal protease-resistant prion protein (PrPres), named high-type (BSE-H) and low-type (BSE-L), that also differed from classical BSE. We characterized 5 atypical BSE-H isolates by analyzing their molecular and neuropathologic properties during transmission in transgenic mice expressing homologous bovine prion protein. Unexpectedly, in several inoculated animals, strain features emerged that were highly similar to those of classical BSE agent. These findings demonstrate the capability of an atypical bovine prion to acquire classical BSE-like properties during propagation in a homologous bovine prion protein context and support the view that the epidemic BSE agent could have originated from such a cattle prion.
• Subjects:
  Animals Atypical BSE Bovine Spongiform Encephalopathy Brain BSE Cattle Encephalopathy, Bovine Spongiform H-type BSE Mice Mice, Transgenic Origin Of BSE Prions And Related Diseases Prion Strain Prion Transmission PrP PrPC Proteins PrPSc Proteins Research Spleen

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• Source:
  Emerg Infect Dis. 17(9):1636-1644.
• Document Type:
  Journal Article
• Volume:
  17
• Issue:
  9
• Collection(s):
  Emerging Infectious Diseases
• Main Document Checksum:
  urn:sha256:923ed04c0532f63aea985768a91f1efdcfc504c13480809f63bbf538eec39a68
• Download URL:
  https://stacks.cdc.gov/view/cdc/23666/cdc_23666_DS1.pdf
• File Type:
  [PDF - 615.69 KB ]