Proteins directly involved in ALS, like TDP-43, SOD1, FUS and the dipeptide repeat proteins derived from C9orf72 aberrant repeats were detected in EVs from in vitro and in vivo models, but no functional role in the disease spread in vivo has been provided yet [14, 15, 54–57]. Here, SOD1 is linked to amyotrophic lateral sclerosis.