This, largely loss-of-function framework also has the potential to exacerbate gain-of-function pathogenic events mediated by the primary pathology underlying the FTLD/ALS disease (TDP-43, FUS, Tau, C9orf72 etc.)and hence the most important molecular pathways affected by hnRNP dysregulation may reflect this pathological heterogeneity. Here, C9orf72 is linked to amyotrophic lateral sclerosis.