Sigurdson
Fakultäten » Medizinische Fakultät » Neuropathologie, Institut für » Prof. Dr. Adriano Aguzzi » Sigurdson
Completed research project
| Title / Titel | Mechanism of prion infectivity generation by genetics | ||
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| Abstract (PDF, 14 KB) | |||
| Summary / Zusammenfassung | Chronic wasting disease (CWD) is a geographically widespread, naturally occurring prion disease of wild and captive North American deer and elk with unknown potential as a human health threat. We have investigated an unusual structural feature of the elk prion protein, a well-defined rigid loop connecting the second strand of the beta sheet with alpha helix 2 (amino acids 165-175), a feature which is not seen in the cow or human prion protein. We have expressed a prion protein mutant in transgenic mice that mimics the elk "rigid loop" (RL). These transgenic mice develop a spontaneous neurologic disease with prion plaques in the brain, similar to deer with CWD or patients with variant Creutzfeldt-Jakob and typical of a transmissible spongiform encephalopathy. We are currently investigating whether this prion disease is transmissible, as this would be the first instance that full length infectious prions have been generated genetically. Therefore the rigid loop transgenic mice serve as a model for familial prion disease in humans. | ||
| Publications / Publikationen | C. Ligios, C.J. Sigurdson, C. Santucciu, G. Carcassola, G. Manco, M. Basagni, C. Maestrale, M.G. Cancedda, L. Madau and A. Aguzzi, PrPSc in mammary glands of sheep affected by scrapie and mastitis. Nat Med 11 (2005) 1137-8. | ||
| Project leadership and contacts / Projektleitung und Kontakte |
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| Funding source(s) / Unterstützt durch |
Forschungskredit der Universität Zürich |
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| Duration of Project / Projektdauer | Nov 2005 to Oct 2007 |