SOD1 and amyotrophic lateral sclerosis: For instance, in amyotrophic lateral sclerosis (ALS), the most common MND, and in the ALS mouse model (SOD1 mutation), blood-spinal cord barrier function of the EC monolayer in the spinal cord breaks down followed by vascular alterations such as insufficient nutrient delivery due to abnormal microvascular structures and leakage of blood components prior to MN degeneration and neurovascular inflammation induced by the loss of endothelial integrity.