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Prion protein devoid of the octapeptide repeat region restores susceptibility to scrapie in prp knockout mice

Academic Article
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  • Identity
  • Additional Document Info
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Overview

authors

  • Flechsig, E.
  • Shmerling, D.
  • Hegyi, I.
  • Raeber, A. J.
  • Fischer, M.
  • Cozzio, A.
  • von Mering, C.
  • Aguzzi, A.
  • Weissmann, Charles

publication date

  • August 2000

journal

  • Neuron  Journal

abstract

  • Mice devoid of PrP are resistant to scrapie and fail to replicate the agent. Introduction of transgenes expressing PrP into such mice restores susceptibility to scrapie. We find that truncated PrP devoid of the five copper binding octarepeats still sustains scrapie infection; however, incubation times are longer and prion titers and protease-resistant PrP are about 30-fold lower than in wild-type mice. Surprisingly, brains of terminally ill animals show no histopathology typical for scrapie. However, in the spinal cord, infectivity, gliosis, and motor neuron loss are as in scrapie-infected wild-type controls. Thus, while the region comprising the octarepeats is not essential for mediating pathogenesis and prion replication, it modulates the extent of these events and of disease presentation.

subject areas

  • Animals
  • Brain Chemistry
  • Brain Tissue Transplantation
  • Caudate Nucleus
  • Ectoderm
  • Fetal Tissue Transplantation
  • Genetic Predisposition to Disease
  • Mice
  • Mice, Knockout
  • Mice, Transgenic
  • Prions
  • Putamen
  • Repetitive Sequences, Amino Acid
  • Scrapie
  • Sequence Deletion
  • Spleen
  • Transgenes
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Identity

International Standard Serial Number (ISSN)

  • 0896-6273

Digital Object Identifier (DOI)

  • 10.1016/s0896-6273(00)00046-5

PubMed ID

  • 10985358
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Additional Document Info

start page

  • 399

end page

  • 408

volume

  • 27

issue

  • 2

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