ALS‐causing mutations in TBK1,31 encoding Tank binding kinase‐1, a protein involved in innate immune system modulation, and the development of autoimmunity in C9ORF72‐null mice32 (C9ORF72 haploinsufficiency being one proposed pathogenic mechanism) support a role for the immune system in ALS pathogenesis, but mechanistic insights into microglial mechanisms are limited. This evidence concerns the gene TBK1 and amyotrophic lateral sclerosis.