SOD1 and amyotrophic lateral sclerosis: Our observations suggesting MN autonomous mechanisms of degeneration in human ALS SOD1 mutants are consistent with previous studies on transgenic mice showing that neuron-specific expression of human mutant SOD1 is sufficient to induce MN degeneration in mice (Jaarsma et al., 2008; Wang et al., 2008), while astrocyte-specific expression of mutant SOD1 did not cause disease (Gong et al., 2000).