Potentially, alterations in tight junction proteins are linked to EC degeneration, implicated in BBB/BSCB breakdown, as we showed in symptomatic G93A SOD1 mutant mice (Garbuzova-Davis et al., 2007a, b) and sporadic ALS patients (Garbuzova-Davis et al., 2012). This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.