Prion family gene involvement in fertility and animal health – PRIFAGENE

The prion protein gene family is composed of three members ; the PrP protein, which has a strong implication in prion diseases, the Doppel protein which is involved in the mouse sperm maturation and the recently identified Shadoo protein whose biological function is yet mostly unknown, as it is also the case for PrP. The expression profiles of these proteins partially overlap suggesting a potential biological complementation and/or cooperation between them explaining the lack of strong phenotypes in studies that are focused on only one of them. This proposal aims at analyzing the potential roles of Shadoo and PrP in early embryogenesis and transmissible encephalopathies and of Shadoo and Doppel in sex determination in mice and small ruminants, three biological aspects with important zootechnical and sanitary implications.