The ISR targeted by protein kinase R (PKR) rescue in a mouse model of FXTAS [143], along with multiple CGG-repeat-associated RBPs, offer potential treatments targeting RAN-translation modifiers, although clinical application awaits further research due to the early stage of Drosophila, cell-based, and mouse experiments. The gene discussed is EIF2AK2; the disease is fragile X-associated tremor/ataxia syndrome.