Since the DNA fragments derived from mtDNA (mt6160) can induce the TDP-43 puncta formation (Fig. S4) and mitochondrial damage has been associated with multiple neurodegenerative diseases, including ALS (61), the cytosolic DNA generated from damaged mitochondria might induce the TDP-43 cytoplasmic puncta. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.