In our study, LPS systemic administration was strongly correlated with an increase in CNS pro-inflammatory microglia and astrocytes (defined as Iba-1+iNOS+ and GFAP +C3+ cells, respectively), and this effect, although present in both SMNΔ7 and control mice, was more prominent for mice with experimental SMA compared to control mice. This evidence concerns the gene GFAP and proximal spinal muscular atrophy.