SOD1 and amyotrophic lateral sclerosis: In familial ALS model mice carrying the mutant SOD1 gene, mutant superoxide dismutase-1 forms aggregates in the ER have been reported to induce the expression of 78 kDa glucose-regulated protein (GRP78/BiP), an ER resident molecular chaperone, and to activate caspase-12, leading to neuronal cell death [7], [8].