EVs have been shown toplay a prominent role in ALS since they could be vehicle of misfolded proteinssuch as TDP-43, FUS, and SOD1 and could represent an optimal biomarker for ALSdiagnosis and/or prognosis, to monitor the disease progression or to develop newtherapeutical approaches [[81], [82], [83]]. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.