Abstract: Amyotrophic lateral sclerosis (ALS) is one of the most dreadful neurodegenerative diseases leading to death within 1–5 years after symptom onset. The majority of ALS cases are sporadic (sALS), while the remaining 5–10% are familial (fALS). Genetic discoveries have identified ALS-causative mutations in more than 30 genes so far (Chia et al., 2018). Indeed, the four most common mutations observed in ALS genes in Europe are the hexanucleotide expansion repeat in Chromosome 9 Open Reading Frame 72 (C9ORF72), Cu-Zn superoxide dismutase 1 (SOD1)), transactive response DNA Binding protein 43kDa (TARDBP) and fused in sarcoma (FUS). These causative genes encode proteins with divergent sub-cellular functions, many of which are associated with defective DNA repair, altered axonal transport, abnormal protein aggregation including impairment of proteostasis and autophagy, mitochondrial dysfunction, and aberrant RNA metabolism (Chia et al., 2018).