The PERK inhibitor GSK2606414 is profoundly neuroprotective in murine models of prion disease [41], and has shown improvement in murine models of Parkinson’s disease [48] and FTD [26] and fly models of amyotrophic lateral sclerosis (ALS) [42], while salubrinal—an inhibitor of eIF2α dephosphorylation—has the opposite effect [24] (see Figure 2). This evidence concerns the gene EIF2A and prion disease.