Our findings reinforce previous data published by other labs, demonstrating that pridopidine exerts neuroprotective effects in numerous models of neurodegenerative diseases including ALS [54], HD [55,82], PD [53], and Alzheimer disease [52], exclusively mediated via activation of the SIGMAR1, and support the ongoing clinical development of pridopidine for the treatment of ALS. Here, SIGMAR1 is linked to Huntington disease.