In yet another genetic mutation of ALS, TDP-43 (TAR DNA-binding protein 43) is commonly found in pathological aggregates in ALS and FTD due to the cytoplasmic accumulation of TDP-43 together with a loss of nuclear TDP-43, thus leading to the proposed disease mechanism involving a loss of normal TDP-43 function in the nucleus, a toxic GOF, or both. Here, TARDBP is linked to frontotemporal dementia.