In the past two decades, increased levels of PKR phosphorylation have been detected in the brains of patients with HIV and neurodegenerative diseases such as Alzheimer’s disease (AD) (Chang et al., 2002), Parkinson’s disease, Huntington’s disease (Peel et al., 2001), dementia, and prion disease (Hugon et al., 2009). Here, EIF2AK2 is linked to juvenile Huntington disease.