In ALS patients, induced pluripotent stem cells (iPSC)-derived motor neurons, TDP-43 mutant mice and NSC-34 cells expressing a doxycycline-induced ALS TDP-43 gene, inflammation is activated through the cGAS/STING pathways after TDP-43 enters mitochondria and triggers mtDNA fragments release via the permeability transition pore [106], identifying mtDNA as a determinant trigger in TDP-43-associated pathology. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.