ALS shares several clinico-pathological features with frontotemporal dementia (FTD) (Lomen-Hoerth et al., 2002; Van Langenhove et al., 2012), including the accumulation and aggregation of aberrant, intracellular inclusions of TAR DNA-binding protein 43 (TDP-43) (Arai et al., 2006; Neumann et al., 2006) that are found in 97% of ALS and 45% of FTD cases (Ling et al., 2013). Here, TARDBP is linked to amyotrophic lateral sclerosis.