This has led to Gcn2 inhibition being explored as a therapeutic approach.51 Alternatively, the group of Ackerman has shown that activation of Gcn2 plays a protective role in animal models of cerebellar and retinal degeneration caused by dual mutations in the ribosome rescue factor GTPBP2 and CNS-specific tRNAArg12, 52 However, the in vivo genetic interaction of Gcn2 with loss of a non-essential tRNA body modification has never been explored. Here, EIF2AK4 is linked to retinal degeneration.