CHMP2B and amyotrophic lateral sclerosis: Other genes, whose mutations are associated with impaired autophagy regulation, are Optineurin (optn) and CHMP2B. OPTN, which is involved in the mitophagy machinery, is proposed to impact on ALS as its mutations promote severe mitochondria degradation (Maruyama et al., 2010; Wong and Holzbaur, 2014).