Among the pathological similarities between ALS and FTLD, one major hallmark is the accumulation of cytoplasmic inclusions of TAR DNA-binding protein 43 (TDP-43) or Fused in Sarcoma/Translocated in Sarcoma (FUS/TLS), which are both ubiquitously expressed in most cell types and tissues. Here, TARDBP is linked to amyotrophic lateral sclerosis.