Fakultäten » Medizinische Fakultät » Neuropathologie, Institut für » Prof. Dr. Adriano Aguzzi » Calella
| Title / Titel | Analysis of the physiological and pathological role of prion protein by proteomic approach in vivo | ||
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| Abstract (PDF, 14 KB) | |||
| Summary / Zusammenfassung | Prion diseases are lethal transmissible spongiform encephalopathies (TSE) affecting many animal species. Prion diseases include Creutzfeldt-Jakob Disease (CJD) in humans, and Bovine Spongiform Encephalopathy (BSE), Scrapie, and Chronic Wasting Disease (CWD) in farm and wild-ranging animals. The central event in prion pathogenesis is the conformational conversion of cellular prion protein (PrPc), a cell surface glycoprotein of unknown function, into PrPSc, an insoluble and protease-resistant isoform, that propagates itself by imposing its abnormal conformation onto PrPc molecules. The precise mechanism of this conversion process, and the nature of the neurotoxic pathways activated by PrPSc, is unknown. I wish to utilize novel transgenetic systems to characterize protein-protein interactions involving PrPC and/or PrPSc, with the aim to clarify the physiological function of PrPc, to identify the molecules chaperoning the conversion of PrPc into PrPSc, and prion neurotoxicity. |
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| Keywords / Suchbegriffe | prions, transmissible spongiform encephalopathies (TSE), Creutzfeldt-Jakob Disease (CJD), neurotoxicity | ||
| Project leadership and contacts / Projektleitung und Kontakte |
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| Funding source(s) / Unterstützt durch |
Foundation Roche Research Foundation |
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| Duration of Project / Projektdauer | Jan 2008 to Jan 2009 |