Since its first description in 2006 in frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS), and over the past decade, the transactive response DNA binding protein of 43 kDa (TDP-43) has been identified as one of the key proteins associated with neurodegenerative diseases, along with amyloid, various strains of tau, and alpha-synuclein, among others (Neumann et al., 2006; Vanden Broeck et al., 2014). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.