Given that the C9ORF72 mutation is the most frequent genetic cause of ALS/FTD and that the majority of ALS patients exhibits the TDP-43 pathology, it could be hypothesized that the enhancement of the paraspeckle formation may contribute to ALS/FTD-related neurodegeneration as a central common neurodegenerative pathway. This evidence concerns the gene TARDBP and frontotemporal dementia.