In this study, we established mSOD1 mice lacking recombination-activating gene 2 (mSOD1/RAG2-/-), an animal model for inherited ALS that lacks mature lymphocytes, and compared their phenotype and microglial characteristics with that of mutant human SOD1 transgenic mice (mSOD1/RAG2+/+). Here, SOD1 is linked to amyotrophic lateral sclerosis.