The undeniable role of the cGAS-STING signaling pathway in innate immune diseases is supported by Al Khatib et al., who demonstrated that TOP1MT influences the activation of the mtDNA-mediated cGAS-STING innate immune response, and that the P193L variant may contribute to the autoimmune phenotype observed in patients (75). The gene discussed is STING1; the disease is immune system disorder.