In amyotrophic lateral sclerosis (ALS), TDP-43 accumulates in the mitochondria of neurons and causes mtDNA release into the cytoplasm via the MPT pore, leading to cGAS-STING activation and the upregulation of NFκB and IFN-I pathways [29]. The gene discussed is NFKB1; the disease is amyotrophic lateral sclerosis.