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)

Molecular Neurobiology

, Volume 8, Issue 2, pp 121-127

First online:

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

  • J. C. MansonAffiliated withInstitute for Animal Health, AFRC and MRC Neuropathogenesis Unit
  • , A. R. ClarkeAffiliated withCRC Laboratories, Department of Pathology, University of Edinburgh
  • , M. L. HooperAffiliated withCRC Laboratories, Department of Pathology, University of Edinburgh
  • , L. AitchisonAffiliated withInstitute for Animal Health, AFRC and MRC Neuropathogenesis Unit
  • , I. McConnellAffiliated withInstitute for Animal Health, AFRC and MRC Neuropathogenesis Unit
  • , J. HopeAffiliated withInstitute for Animal Health, AFRC and MRC Neuropathogenesis Unit

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Abstract

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