In the case of ALS, our experimental approach consisted on generating a double mutant mouse model having the partial deficiency in BiP protein and the G93A mutant form found in the human SOD1 gene, whose behavioral and histopathological abnormalities were compared first with mSOD1 mice and also with the two control genotypes (wildtype or BiP+/− mice). This evidence concerns the gene HSPA5 and amyotrophic lateral sclerosis.