Four independent studies have provided evidence that C9orf72 HRE induces dysfunctional nucleocytoplasmic trafficking in ALS/FTD, implying that the C9orf72 HRE also perturbs protein quality control (Freibaum et al., 2015; Jovičić et al., 2015; Zhang et al., 2015a; Woerner et al., 2016). Here, C9orf72 is linked to amyotrophic lateral sclerosis.