Still asymptomatic 10-week-old FUS (1-358) (Shelkovnikova et al. 2013) and 12-week-old SOD1 (G93A) mutant mice (de Munter et al. 2019b) were treated with bm-SC transplantations before developing a progressive loss of motor functions, muscle atrophy and weight loss due to a fast degeneration of spinal motor neurons with denervation in the following weeks, thereby representing a relevant preclinical model for amyotrophic lateral sclerosis (ALS). This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.