Norwegian Dairy Goats Naturally devoid of the prion protein - a break-through in the battle against prions?

Gjennom prosjektet er det avdekket at geiter uten PrP har en sterkere sykdomsrespons etter inflammasjon utløst av bakterielle endotoksiner (LPS). Dette er satt i sammenheng med ulik følsomhet i flere signalveier i medfødt immunitet, spesielt Type 1 interferon. Våre funn tyder på at PrP har en modulerende rolle i disse signalveiene, som ikke har latt seg oppdage i musemodeller innen prionforskningen. Våre funn har pekt ut nye retinger i studiene av PrPs normalfunksjon, som kan være en stor betydning for vår forståelse av prionsykdommer og andre lignende nevrodegenerative lidelser knyttet til proteinavleiringer.

Research on prion diseases, such as Creutzfeldt-Jakob disease and scrapie, has identified the prion protein (PrP) as the culprit of these deadly brain disorders. Large bodies of experimental evidence, dictates that any human or animal without endogenous e xpression of PrP would be completely resistant towards prion infection, since prion-replication would not occur. Prion diseases continue to pose a significant threat against human and animal health and costly preventive measures have been implemented glo bally to combat this. Beyond food-safety, the bio-safety of medicals is also a major concern, since prions are difficult to detect and almost impossible to remove/inactivate while maintaining desired product qualities. Thus, prion-free resources for biome dicals are sought for. We have recently discovered a novel line of Norwegian goats that carry a mutation that prevents expression of PrP, while leaving the goats apparently healthy. In this proposal we focus on this significant discovery by asking the fo llowing questions; a) Are these goats completely prion resistant? b) Do goats with the PrP mutation carry with them recognizable additional traits? c) Are these goats suited for breeding and production? In order to address these questions we have designed a series of studies in co-operation with the Norwegian Goat Breeders association (NSG) and the goat health monitoring body of Norway (HT-goat/TINE). We think that this project carries with it highly significant socio-economic potential for Norwegian goat production and related industries in and outside Norway.