Dysregulated PERK/eIF2α signaling during the UPR is a common underlying mechanism of neurodegenerative diseases, including AD, PD, Huntington’s disease (HD), amyotrophic lateral sclerosis, etc. (Hetz and Mollereau, 2014; Bell et al., 2016; Ohno, 2018). Here, EIF2AK3 is linked to juvenile Huntington disease.