Publication
Prion protein devoid of the octapeptide repeat region restores susceptibility to scrapie in PrP knockout mice
Journal Paper/Review - Aug 1, 2000
Flechsig E, Shmerling D, Hegyi I, Raeber A J, Fischer M, Cozzio Antonio, von Mering C, Aguzzi A, Weissmann C
Units
PubMed
Citation
Type
Journal
Publication Date
Issn Print
Pages
Brief description/objective
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.