Remarkably, the knock-in mice that express mutant GRP78 lacking the KDEL sequence have age-related motor problems concomitant with loss of selective vulnerable motoneurons and aggregation of wild-type SOD1 reminiscent of ALS symptoms (Bosco et al., 2010; Jin et al., 2014). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.