Although we show here that the Sig-1R with a single amino acid mutation at 102 has a reduced ability to bind (G4C2)10-RNA (Fig. 4d), we do not know if this mutation of Sig-1Rs plays a role in the (G4C2)-RNA repeats-induced ALS/FTD. Here, SIGMAR1 is linked to amyotrophic lateral sclerosis.