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Expression of amino-terminally truncated prp in the mouse leading to ataxia and specific cerebellar lesions

Academic Article
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Overview

authors

  • Shmerling, D.
  • Hegyi, I.
  • Fischer, M.
  • Blattler, T.
  • Brandner, S.
  • Gotz, J.
  • Rulicke, T.
  • Flechsig, E.
  • Cozzio, A.
  • von Mering, C.
  • Hangartner, C.
  • Aguzzi, A.
  • Weissmann, Charles

publication date

  • April 1998

journal

  • Cell  Journal

abstract

  • The physiological role of prion protein (PrP) remains unknown. Mice devoid of PrP develop normally but are resistant to scrapie; introduction of a PrP transgene restores susceptibility to the disease. To identify the regions of PrP necessary for this activity, we prepared PrP knockout mice expressing PrPs with amino-proximal deletions. Surprisingly, PrP lacking residues 32-121 or 32-134, but not with shorter deletions, caused severe ataxia and neuronal death limited to the granular layer of the cerebellum as early as 1-3 months after birth. The defect was completely abolished by introducing one copy of a wild-type PrP gene. We speculate that these truncated PrPs may be nonfunctional and compete with some other molecule with a PrP-like function for a common ligand.

subject areas

  • Alleles
  • Animals
  • Ataxia
  • Brain Chemistry
  • Cell Death
  • Cerebellum
  • Genes
  • Mice
  • Mice, Transgenic
  • Neurons
  • Phenotype
  • Prions
  • RNA, Messenger
  • Scrapie
  • Sequence Deletion
  • Time Factors
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Identity

International Standard Serial Number (ISSN)

  • 0092-8674

Digital Object Identifier (DOI)

  • 10.1016/s0092-8674(00)81572-x

PubMed ID

  • 9568713
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Additional Document Info

start page

  • 203

end page

  • 214

volume

  • 93

issue

  • 2

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