We demonstrated an increase of S100A4 expression in microglia and astrocytes of the spinal cord from transgenic superoxide dismutase 1 (SOD1)-G93A rats and in fibroblasts derived from ALS patients carrying SOD1 pathogenic variants, indicating a specific cell type overexpression of S100A4 and suggesting its possible inflammatory function in ALS. Here, S100A4 is linked to amyotrophic lateral sclerosis.