Abstract
Firstly, I will briefly recapitulate the history of prion diseases and the development of the protein only (prion) hypothesis. Then, move on to the recent observations of prion diseases in Norwegian reindeer and moose and touch upon some of the ongoing research connected to this. The disease features are strikingly different when comparing the outbreak in Nordfjella wild reindeer and the cases in moose. This will be discussed in view of similar observations in small ruminant scrapie and human prion diseases. Finally, data from a novel spontaneous animal model for PrP research will be presented. In 2012, a nonsense mutation at codon 32 of the goat PRNP gene was discovered and animals homozygous for this mutation are devoid of PrP. We have studied this unique animal model, looking at hematological and immunological parameters and responses to acute LPS-induced inflammation. In line with data from Tg mice models, subtle phenotypes were observed. Finally, recent observations of a clinically silent demyelinating peripheral polyneuropathy in goats without PrP will be discussed.
Michael A. Tranulis
Norwegian University of Life Sciences, Faculty of Veterinary Medicine, Oslo, Norway
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