Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are characterized pathologically by the accumulation of cytoplasmic inclusions in affected neurons and glial cells that, in 97%–98% of cases, stain positive for TAR-DNA binding protein 43 (TDP-43; Neumann et al., 2006). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.