This characteristic high RNA concentration is reported to prevent aggregation of prion-like RNA binding proteins (RBPs) such as FUS and TDP43 in the nucleus [62,63], while FUS and TDP43 tend to aggregate in the cytoplasm probably due to less RNA concentration, causing neurodegenerative diseases including frontotemporal dementia and amyotrophic lateral sclerosis [62,64]. Here, TARDBP is linked to frontotemporal dementia.