Inclusions of the RNA-binding protein TDP-43 have been identified as the main hallmarks of amyotrophic lateral sclerosis (ALS) (Mackenzie et al, 2007), setting in motion a tide of studies showing that TDP-43 inclusions are also the primary features of additional conditions including subtypes of frontotemporal dementia and even traumatic brain injury. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.