Yu et al. used transgenic mice with the TDP-43 mutant allele A315T, STING-deficient mice, and the resulting progeny from crossing these two lines to demonstrate that TDP 43 releases mtDNA through the mPTP permeability pore and activates the cGAS/STING pathway thereby exacerbating ALS [85]. The gene discussed is STING1; the disease is amyotrophic lateral sclerosis.