Although specific causes leading to ALS are unknown, different cellular mechanisms were proposed to mediate MN degeneration, such as glutamate excitotoxicity, mitochondrial dysfunction, protein aggregation, proteasomal and autophagic dysfunction, neuroinflammation, altered axonal transport, and impaired RNA metabolism.1 In this context, the role of alterations of RNA metabolism seems particularly central, especially considering that TDP43 and FUS are key components of coding and noncoding RNA processing. This evidence concerns the gene FUS and amyotrophic lateral sclerosis.