Miele Genoud
Fakultäten » Medizinische Fakultät » Neuropathologie, Institut für » Prof. Dr. Adriano Aguzzi » Miele Genoud
Completed research project
| Title / Titel | Identification of genes differentially expressed in testes of Prion protein and Doppel double knockout mice | ||||||
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| Abstract (PDF, 14 KB) | |||||||
| Summary / Zusammenfassung | Expression of Prion protein (PrPC) is an absolute requirement for pathogenesis of prion diseases. However, at present the precise physiological function of PrPC remains elusive and whilst several lines of Prnp-/- mice have been generated, these mice have not yielded useful clues to the physiological function of PrPC so far. It is a strong possibility that there may be redundancy of PrPC and that functional compensation may occur in these mice, whereby the function of PrPC can be assumed by another protein in its absence. Recently, an open reading frame has been discovered which encodes a protein (Doppel, Dpl) that shares significant structural homology with PrPC. Dpl is expressed at high levels in testis and ablation of Dpl (Prnd-/-) results in male sterility. Significantly, transgenic expression of PrPC is able to rescue ataxic phenotypes associated with ectopic Dpl expression in the CNS, which is highly suggestive that PrPC and Dpl function in a common/overlapping pathway. To abrogate full or partial functional compensation of Dpl in testis of Prnp-/- mice, we have generated mice deficient for both PrPC and Dpl. These mice represent a unique opportunity with which to investigate and gain clues to the normal physiological function of PrPC and Dpl. We are therefore utilising these mice in microarray studies to identify genes which are differentially expressed as a direct result of the ablation of these proteins. |
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| Keywords / Suchbegriffe | Doppel, PrP, Prnd, Prnp, microarray, gene expression | ||||||
| Project leadership and contacts / Projektleitung und Kontakte |
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| Other links to external web pages | http://www.uzh.ch/pathol/neuropathologie/d/index.html | ||||||
| Funding source(s) / Unterstützt durch |
Others UK Biotechnology & Biological Sciences Research Council (Gino Miele) |
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| Duration of Project / Projektdauer | Jan 2003 to Dec 2005 |