In nearly all cases of amyotrophic lateral sclerosis (ALS), TDP-43 mislocalises to the cytoplasm and forms insoluble aggregates in neurons and glia; similar pathology occurs in ∼45% of frontotemporal dementia (FTD), limbic-predominant age-related TDP-43 encephalopathy (LATE) and in Alzheimer’s disease co-morbid with LATE-NC (LATE-neuropathological changes) (Arai et al, 2006; Neumann et al, 2006; Mackenzie et al, 2007; Josephs et al, 2014; Nelson et al, 2019). Here, TARDBP is linked to frontotemporal dementia.