Molecular Neurobiology

, Volume 8, Issue 2–3, pp 105–112 | Cite as

PrP in pathology and pathogenesis in scrapie-infected mice

  • M. E. Bruce
  • P. A. McBride
  • M. Jeffrey
  • J. R. Scott
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)


PrP accumulation in the brains of mice infected with scrapie takes several different forms: amyloid plaques, widespread accumulation in neuropile, and perineuronal deposits. PrP is also sometimes detected within microglia and in or around astrocytes. There are dramatic and reproducible differences between scrapie strains in the relative prominence of these changes and their distribution in the brain. Depending on the scrapie strain, PrP pathology is targeted precisely to particular brain areas, often showing a clear association with identifiable groups of neurons. These results suggest that PrP changes are primarily associated with neurons, and that different scrapie strains recognize and selectively replicate in different populations of neurons. Immunostaining at the ultrastructural level demonstrates an association of PrP with neurite plasmalemma, around amyloid plaques, and in areas of widespread neuropile and perineuronal accumulation. It is probable that PrP is encoded by theSinc gene, which controls the incubation period of scrapie in mice. Studies using the intraocular infection route show that theSinc gene controls the onset rather than the rate of replication, suggesting that PrP may be involved in cell-to-cell spread of infection. The accumulation of PrP at the surface of neurons is consistent with such a role.

Index Entries

Scrapie PrP scrapie strains scrapie pathology scrapie pathogenesis, mouseSinc gene 


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Copyright information

© Humana Press Inc 1994

Authors and Affiliations

  • M. E. Bruce
    • 1
  • P. A. McBride
    • 1
  • M. Jeffrey
    • 2
  • J. R. Scott
    • 1
  1. 1.IAH AFRC & MRC Neuropathogenesis UnitEdinburghUK
  2. 2.Lasswade Veterinary LaboratoryPenicuikUK

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