The significance of changes in the expression levels of SigR1 in ALS has been inconsistently reported; expression levels of mutant Sig1R proteins (c672*51G > T) are either elevated in leukocytes and the frontal cortex [120] or not different in primary lymphoblastoid cells derived from patients with ALS carrying mutant Sig1RE102Q [121]. Here, SIGMAR1 is linked to amyotrophic lateral sclerosis.