In light of these findings, multiple recent studies have associated the activation of the mtDNA-cGAS–STING pathway, or more broadly, aberrant innate immune signaling, with the development of some neurodegenerative pathologies, such as amyotrophic lateral sclerosis (ALS) or Huntington’s disease (HD) [51,87]. This evidence concerns the gene CGAS and amyotrophic lateral sclerosis.