Based on our previous findings of the protective role of TDP-43 on UGGAA repeat-induced toxicity in spinocerebellar ataxia type 31 (SCA31) models (Ishiguro et al., 2017), we hypothesized that RBPs specifically binding to repeat sequences of template RNA play a role in RAN translation. The gene discussed is RAN; the disease is spinocerebellar ataxia type 31.