In support of a neuroprotective role for Dorfin in ALS, Dorfin ubiquitinates ALS-associated mutant SOD1 (superoxide dismutase 1) protein33, and, when overexpressed in a mouse model of familial ALS, reduces the amount of mutant SOD1 proteins and suppresses neurological phenotypes and motor neuron death40. Here, SOD1 is linked to amyotrophic lateral sclerosis.