Co-localization and interaction of CHGC and B with mutant SOD1 was seen in ALS mice [31], and interestingly, also interaction of oxidized wild type SOD1 with CHGB and subsequent motor neuron death has been observed [46,47] A variant in Chromogranin B has been identified as disease modifier of mutant SOD1-ALS and in one study also of sporadic ALS in a Canadian cohort [34,48]. Here, CHGB is linked to amyotrophic lateral sclerosis.