Recently, it has been demonstrated that the intramitochondrial TDP-43-induced production of mtROS also triggers the opening of the mitochondrial permeability transition pore and leakage of mitochondrial DNA into the cytoplasm, resulting in the activation of the cyclic guanosine monophosphate-adenosine monophosphate synthase (cGAS)/stimulator of interferon genes (STING) pathway in ALS patient iPSC-derived motor neurons and the spinal cords of transgenic mice expressing ALS-associated TDP-43 [90]. Here, STING1 is linked to amyotrophic lateral sclerosis.