Super oxide dismutase 1 (SOD1) was the first ALS gene to be identified in 1993, since then more than 120 genetic variants have been associated with a risk of ALS and at least 25 of these genes have been reproducibly implicated in familiar ALS with moderated penetrance, but nowadays 80% of familial cases are not linked to known genetic causes [5]. Here, SOD1 is linked to amyotrophic lateral sclerosis.