Original data demonstrating altered expression of Ca2+ transporters and disturbed local Ca2+ fluxes in primary cultures of SMs from hSOD1(G93A) Tg mice indicate that such alterations may be responsible for early SM dysfunctions in ALS, twisting the interplay between SMs and MNs and contributing to MN death. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.