Aguzzi
Fakultäten » Medizinische Fakultät » Neuropathologie, Institut für » Prof. Dr. Adriano Aguzzi » Aguzzi
Completed research project
| Title / Titel | C5 Function of the cellular prion protein PrPC, and of its homologue, Dpl | ||||
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| Abstract (PDF, 14 KB) | |||||
| Summary / Zusammenfassung | The prion protein PrPC is essential for the development of prion diseases, but its physiological function remains unknown. It is important to clarify the latter, since it is likely to impinge on the sequence of events that leads to prion neurotoxicity. Mice devoid of PrPC do not suffer from se-vere phenotypes, yet some amino proximally truncated versions of PrPC (ΔPrP) can induce se-vere neurodegeneration when expressed transgenically in mice lacking PrPC. These defects are rescued by co-expression of full-length PrPC, providing a genetic model to investigate PrPC de-pendent neural functions. The recently discovered prion protein homologue Doppel (Dpl) is structurally similar to ΔPrP and, when overexpressed, also causes neurodegenerative changes. Here we propose to dissect the neurological diseases induced by ΔPrP and by Dpl using tools of morphology, molecular and structural biology, with the aim to better understand the role of the PrP/Dpl protein complexes in the brain. | ||||
| Publications / Publikationen | Radovanovic, I., Braun, N., et al. (2005) “Truncated prion protein and Doppel are myelinotoxic in the absence of oligodendrocytic PrPC” J. Neurosci. 25 p4879 fBaumann et al. (2006) “Lethal recessive myelin toxicity of prion protein lacking its central domain” submitted to EMBO J.Baumann F. „New Insights into PrPC-functional domains” Presentation at II In-ternational Symposium on “The new prion biology: basic science, diagnostis and therapy” 7.-9.4.2005 Venice (Italy) | ||||
| Keywords / Suchbegriffe | Prion diseases, Cellular Biology, Neurobiology | ||||
| Project leadership and contacts / Projektleitung und Kontakte |
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| Funding source(s) / Unterstützt durch |
SNF (Personen- und Projektförderung), Other Public Sources (e.g. Federal or Cantonal Agencies) |
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| Duration of Project / Projektdauer | Jul 2003 to Jun 2007 |