Mechanically, mislocalized mitochondrial TDP43 causes mtDNA release through mitochondrial permeability transition pore (MPTP) opening and leakage through VDAC1, resulting in the cGAS-STING-dependent induction of type I interferons and inflammatory cytokines.211 Expansions of a repeat hexanucleotide (GGGGCC) in the C9orf72 gene are the alternative cause of familial ALS and FTD. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.