It examines the most recent evidence regarding the involvement of ALS-associated mutant genes in immune dysregulation, focusing on the cyclic GMP-AMP synthase (cGAS)-stimulator of interferon genes (STING) signaling pathway and N6-methyladenosine (m6A)-mediated immune regulation in the context of neurodegeneration. This evidence concerns the gene CGAS and amyotrophic lateral sclerosis.