Our findings show that FMRpolyG, in the absence of CGG mRNA, causes both cell death and lamin ring disruption, two important features of cellular toxicity observed in previous model systems for FXTAS (Arocena et al., 2005; Hoem et al., 2011; Sellier et al., 2017). This evidence concerns the gene LMNA and fragile X-associated tremor/ataxia syndrome.