Moreover, ALS pathogenesis also progresses without axonal neurofilaments [57], and over-expression of NEFH prolongs survival of SOD1-transgenic FALS mice, prompting the hypothesis that NFs primarily act as an abundant buffer for otherwise deleterious processes, such as actions of SOD1 gain-of-function mutants [58] or aberrant tyrosine nitrosylation or phosphorylation [1,59,60]. Here, SOD1 is linked to amyotrophic lateral sclerosis.