Increased levels of phosphorylated PKR have been reported in the brains of Alzheimer’s disease (AD) patients (Chang et al., 2002), Parkinson’s disease, Huntington’s disease (Peel et al., 2001; Peel and Bredesen, 2003), dementia (Taga et al., 2017), and prion disease (Paquet et al., 2009). Here, EIF2AK2 is linked to prion disease.