Abnormal cytoplasmic accumulations of TDP-43 are a common occurrence in degenerating neurons of the CNS in the majority of ALS cases and of tau-negative frontotemporal dementia (FTD) as well as in subsets of Alzheimer's disease and Parkinson's disease (Scotter et al., 2015). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.