Based on these analyses in human ALS and the accumulating evidence that microglia are dysfunctional early in the SOD1G93A murine model, which may promote/propagate astrocytic and neuronal dysfunction and that RAGE is known to regulate in vitro microglia-like cell responses to several ligands increased in SOD1G93A mice [25, 33, 34, 70–73], we sought to examine if microglia expressed RAGE in a prototypic murine model of ALS, the SOD1G93A model. The gene discussed is AGER; the disease is amyotrophic lateral sclerosis.