ALS/FTD-causing mutations within or adjacent to the RRMs (P112H, D169G, K181E, K263E) reduce RNA binding and increase aggregation propensity of TDP-43 [220–222], similar to introducing lysine acetylation mimics in RRM1 (K136, K145) and RRM2 (K192) [210, 219, 223, 224]. Here, TARDBP is linked to frontotemporal dementia.