PRNP and human prion disease: These diseases, called in general Transmissible Spongiform Encephalopathy (TSE), include Bovine Spongiform Encephalopathy (BSE or mad cow disease), Scrapie in sheep, Creutzfeldt–Jakob disease and Kuru in humans [1,2], etc. These diseases are caused by aggregation of the prion protein PrP in the amyloid form, which is a product of the PRNP gene in humans [3,4,5,6].