Therefore, taking into account that we have found an induction in TGF-β signaling, together with an increase in ECM molecules deposition in skeletal muscle of symptomatic hSOD1G93A mice, we speculate that targeting TGF-β and other pro-fibrotic factors may be a suitable therapeutic approach to restore, at least partially, muscle performance in ALS patients. This evidence concerns the gene TGFB1 and amyotrophic lateral sclerosis.