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Japanese scrapie cases.

T Onodera1, K Saeki

  • 1Department of Molecular Immunology, School of Agricultural and Life Sciences, University of Tokyo, Tokyo 113-8657, Japan. aonoder@mail.ecc.u-tokyo.ac.jp.

Japanese Journal of Infectious Diseases
|June 29, 2000
PubMed
Summary
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Scrapie, a transmissible spongiform encephalopathy, was experimentally transmitted to sheep from cattle with bovine spongiform encephalopathy. Sheep-to-sheep transmission is likely, with linked prion protein gene expression potentially causing neurodegenerative disorders.

Area of Science:

  • Neuroscience
  • Veterinary Medicine
  • Genetics

Background:

  • Bovine spongiform encephalopathy (BSE) can be transmitted to sheep, presenting clinically as scrapie.
  • Scrapie cases in Japan, particularly in Suffolk and Corriedale breeds, suggest potential sheep-to-sheep transmission.
  • Normal prion protein (PrP) is implicated in cellular signaling, and its overexpression can lead to neurodegeneration.

Purpose of the Study:

  • To investigate the potential for sheep-to-sheep transmission of scrapie.
  • To explore the role of prion protein (PrP) and its related gene, Prnd, in neurodegenerative disorders.

Main Methods:

  • Clinical observation and experimental transmission studies of scrapie in sheep.
  • Genetic analysis of prion protein gene (Prnp) and Prnd expression in affected animals and cell lines.

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Main Results:

  • Experimental transmission of BSE to sheep resulted in a condition clinically indistinguishable from scrapie.
  • Evidence suggests sheep-to-sheep transmission of scrapie occurred in Hokkaido, Japan.
  • Doppel (Dpl), a protein encoded by Prnd, was upregulated in PrP-deficient models and caused neurodegeneration.

Conclusions:

  • Sheep-to-sheep transmission of scrapie is a significant concern.
  • The linked expression of Prnp and Prnd may contribute to the pathogenesis of various neurodegenerative diseases.