As an example, the cytoplasmic and mitochondrial accumulation of TAR DNA-binding protein 43 (TDP-43, whose mutations are associated with amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)) determines the release of mtDNA (one of the best-characterized mtDAMPs [36]) in the cytoplasm. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.