Cytoplasmic inclusions of phosphorylated TAR DNA-binding protein-43 (TDP-43) are a pathological hallmark of several neurodegenerative disorders including amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD) and limbic-predominant age-related TDP-43 encephalopathy (LATE) [6, 20, 48, 53, 66–68, 81]. The gene discussed is TARDBP; the disease is frontotemporal dementia.