In many neurodegenerative diseases, including frontotemporal dementia, Alzheimer’s disease (AD), amyotropic lateral sclerosis (ALS), and multiple sclerosis (MS), cytoplasmic mislocalization and aberrant aggregation of TDP-43 have been observed in affected regions of the CNS (Neumann et al., 2006; Ling et al., 2015; Sun et al., 2017; Masaki et al., 2020; Salapa et al., 2020). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.