Although the molecular pathogenesis of TSEs is still not well understood, it is widely accepted that the conformational transition of the native and predominantly α-helical cellular prion protein (PrPC) to a β-sheet rich pathogenic scrapie isoform (PrPSc) that results in the accumulation of PrPSc aggregates is the central step/event of the diseases. Here, PRNP is linked to scrapie.