Loss of RCC1 (also known as RanGEF) enhanced toxicity of PR50 in S.cerevisae (Jovičić et al., 2015), and nuclear intensity of RCC1 was decreased in iPSC-derived neurons from C9orf72-ALS cases (Jovičić et al., 2015). The gene discussed is RCC1; the disease is amyotrophic lateral sclerosis.