Mutations in several genes coding for proteins involved in autophagy or other membrane trafficking pathways have been found in ALS patients, including SQSTM1 and OPTN, which encode the selective autophagy receptors SQSTM1/p62 and optineurin, respectively, and ALS2 that encodes alsin2, which participates in membrane trafficking, as well as TBK1 (TANK-binding kinase 1), a kinase involved in autophagy-mediated degradation of ubiquitinated cargos [17–19]. Here, OPTN is linked to amyotrophic lateral sclerosis.