SIGMAR1 and amyotrophic lateral sclerosis: Despite of these facts, it should also be noted that our findings in Sig1R−/− mice such as MAM disruption and Sig1R aggregation are well consistent with the pathological features reported in sporadic ALS cases in human (Prause et al, 2013), and therefore, Sig1R−/− mice are the useful tool to examine the molecular pathomechanism of ALS.