Linkage analysis of patients affected with multiple cases of ALS, FTD, and FTD-ALS with type 2 TDP-43 pathology had suggested that there was an important locus for the disease on chromosome 9p [18–21], but it is not clear whether the linkage and association signals related to a single locus or whether the different studies are reporting the same alleles at that locus. Here, TARDBP is linked to amyotrophic lateral sclerosis.