While we observe significant neuromuscular defects that reflect a characteristic distal-to-proximal ALS disease course, there are still unanswered questions that should be addressed to understand why the disease course is not as severe in zebrafish as it is in G93A-SOD1 rodents, and why drastic locomotor deficits are not observed even in the context of MN loss. Here, SOD1 is linked to amyotrophic lateral sclerosis.