In a comprehensive study [123] using induced pluripotent stem cell (iPSC)-derived motor neurons and TDP-43 mutant mice, it was demonstrated that TDP-43 causes inflammation in ALS by triggering the release of mitochondrial DNA into the cytoplasm, subsequently activating the cGAS/STING pathway, and resulting in neuroinflammation. Here, TARDBP is linked to amyotrophic lateral sclerosis.