It has been reported that accumulation of ubiquitin- and SQSTM1-positive aggregates is observed not only in ALS patients but also in a SOD1H46R ALS mouse model [22], and that proliferation of astrocytes and the activation of microglia also occur in SOD1H46R mice as disease progresses. The gene discussed is SQSTM1; the disease is amyotrophic lateral sclerosis.