These diseases are characterized by extracellular deposition of partially protease-resistant PrP aggregates (termed scrapie prion protein, or PrPSc) within the central nervous system (CNS), sometimes in form of amyloid plaques, accompanied by conspicuous neuronal loss and vacuolation and by pronounced astrogliosis and microgliosis [1]. Here, PRNP is linked to amyloidosis.