The exact mechanism by which SOD1 mutations lead to ALS pathology is unknown although numerous hypotheses have been proposed to explain SOD1-mediated toxicity such as misfolding proteins-associated aggregation, oxidative stress, mitochondrial dysfunction, endoplasmic reticulum stress, glutamate excitotoxicity, inflammation and microglial activation and axonal transport abnormalities[2,5]. Here, SOD1 is linked to amyotrophic lateral sclerosis.