Furthermore, MITOL seems to be involved in the ubiquitination and degradation of misfolded proteins located in mitochondria, such as a mutated form of superoxide dismutase 1 (SOD1), an antioxidant enzyme that has been implicated in amyotrophic lateral sclerosis (ALS, or Lou Gehrig’s disease) [79]. The gene discussed is MARCHF5; the disease is amyotrophic lateral sclerosis.