PRNP and Creutzfeldt Jacob disease: Outside of Kuru and CJD, other human pathologies termed transmissible spongiform encephalopathies have been linked to the misfolding of PrP [110], namely the variant CJD (the “mad cow” disease) [111], Gerstmann–Sträussler-Scheinker disease [112], fatal familial insomnia [113] and variably protease-sensitive prionopathy [114].