C9orf72 and amyotrophic lateral sclerosis: Strikingly, we discovered that neuroprotection is conferred by manipulating the expression of a small proportion of C9ORF72-ALS-altered transcripts (362 transcripts genome-wide) in addition to inhibiting the nuclear export of pathological C9ORF72-repeat transcripts and DPR-associated neurotoxicity, known to cause widespread alteration of gene expression through disruption of the nucleolus [57], splicing [54, 55] and nucleocytoplasmic transport of proteins [56, 59] although potentially indirectly [58].