Molecular Neurobiology

, Volume 8, Issue 2–3, pp 121–127 | Cite as

129/Ola mice carrying a null mutation in PrP that abolishes mRNA production are developmentally normal

  • J. C. Manson
  • A. R. Clarke
  • M. L. Hooper
  • L. Aitchison
  • I. McConnell
  • J. Hope
Neurodegenerative Diseases Part II: Transmissible Neurodegenerative Disorders (Proceedings of the symposium “Transmissible and Nontransmissible Neurodegenerative Disorders” held in Ocho Rios, Jamaica, February 28–March 5, 1993)


The neural membrane glycoprotein PrP is implicated in the pathogenesis of the transmissible spongiform encephalopathies; however, the normal function of PrP and its precise role in disease are not understood. Recently, gene targeting has been used to produce mice withneo/PrP fusion transcripts, but no detectable PrP protein in the brain (1). Here we report the use of a different targeting strategy, to produce inbred mice with a complete absence of both PrP protein and mRNA sequences. At 7 mo of age, these mice show no overt phenotypic abnormalities despite the normal high levels of expression of PrP during mouse development. The mice are being used in experiments designed to address the role of PrP in the pathogenesis of scrapie and the replication of infectivity.

Index Entries

Gene targeting PrP gene null mutation 


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Copyright information

© Humana Press Inc 1994

Authors and Affiliations

  • J. C. Manson
    • 1
  • A. R. Clarke
    • 2
  • M. L. Hooper
    • 2
  • L. Aitchison
    • 1
  • I. McConnell
    • 1
  • J. Hope
    • 1
  1. 1.Institute for Animal HealthAFRC and MRC Neuropathogenesis UnitEdinburgh
  2. 2.CRC Laboratories, Department of PathologyUniversity of EdinburghEdinburghUK

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