HSPA5 and amyotrophic lateral sclerosis: In the present study, we investigated the involvement of a dysregulation of BiP in the pathogenesis of ALS by using mSOD1(G93A) transgenic mice, which were crossed with mice harboring a partial genetic deletion of the BiP gene (BiP+/− mice) (Note that very early embryonic lethality occurs in BiP−/− mice [26]) to generate double mutants (mSOD1/BiP+/− mice), in which we analyzed the progression of the pathological phenotype.