A deadly prion-borne disease, Chronic Wasting Disease (CWD) has been detected in Scandinavian deer, 19 wild reindeer and 4 moose in Norway 2016, one moose in Finland 2018 and at three moose in Sweden 2019. CWD is a transmissible spongiform -cephalopathy (TSE) as well as Scrapie in sheep, BSE in cows and Creutzfeldt-Jacobs disease in humans.
The prion protein (PrP) is a body protein in mammals that in altered form gives rise to disease in humans and animals. As the PrP structure changes and aggregates, very stable infectious prions forming fibrous filaments, amyloids are formed. The folding form is stable and self-replicates in a catalyzed process and transforms the healthy PrP into the diseased form. Norwegian researchers have discovered that the Norwegian CWD cases differ from those occurring in North America and that CWD in Scandinavian deer animals differ among themselves.
For Sweden and the Nordic countries, knowledge about prions is limited and very little research has been done in this area in our part of the world. With the findings of CWD made in Scandinavia, EU has decided on a monitoring of moose and reindeer. Monitoring is important for detecting cases of the disease and information is important for epidemiological analyzes. But ONLY monitoring is not sufficient for understanding the prevalence, spread and source of prion diseases, major research efforts are needed.
SLU has shown that genetic variations in the body protein PrP are of great importance for the susceptibility of infection as well as for the spread of infection within or between species. At the Dept. of Anatomy, Physiology and Biochemistry, research is being conducted on developing sensitive methods for detecting prions. Movement ecology of ungulates is an important research topic at the Dept. of Wildlife, Fish, and Environmental Studies. One of the moose diagnosed with CWD in Sweden had a GPS (global positioning system) collar and analysis of movement of thisand other diseased animals can give important insights on potential contamination and risk areas.