Raised levels of PERK-P and eIF2α-P are observed in prion-diseased mice (Unterberger et al., 2006; Moreno et al., 2012; Halliday et al., 2015); in Tg4510 mice, which overexpress the frontotemporal dementia-associated human tau mutation P301L (Abisambra et al., 2013; Radford et al., 2015); in 5xFAD mice that express five Alzheimer’s disease-linked mutations (Devi and Ohno, 2014); and in mutant SOD1-expressing mice (Saxena et al., 2009). This evidence concerns the gene SOD1 and early-onset autosomal dominant Alzheimer disease.