However, a previous study showed that NUP54 can restore the proper nuclear localization of TDP-43 by alleviating the negative nuclear transport effects caused by another poly-dipeptide protein, poly-GA (Khosravi et al. 2017), suggesting that NUP54 has paradoxical pathological mechanisms through interactions with multiple poly-dipeptides in ALS (Fig. 4, Table 3). This evidence concerns the gene NUP54 and amyotrophic lateral sclerosis.