TARDBP and frontotemporal dementia: TDP-43 loss from the nucleus and its mislocalisation to the cytoplasm in the CNS cell types, sometimes accompanied by its aggregation (‘TDP-43 proteinopathy’), are observed in nearly all patients with sporadic amyotrophic lateral sclerosis (ALS), a large proportion of familial ALS and frontotemporal dementia (FTD), a subset of Alzheimer's disease, multisystem proteinopathy and myopathy cases [105,106].