A (GGGGCC)n expansion, of >30 repeat units, in intron 1 of chromosome 9 open reading frame 72 (C9ORF72) causes C9ORF72 FTD/ALS, whereas SCA36 is caused by a (TGGGCC)n expansion of 650 or more repeats in intron 1 of the nucleolar protein 56 (NOP56) [88,89,90,91] (Figure 1). Here, NOP56 is linked to amyotrophic lateral sclerosis.