Optineurin (OPTN), an autophagy receptor causative for glaucoma and ALS (Maruyama and Kawakami, 2013; Maruyama et al., 2010; Rezaie et al., 2002), is recruited to the mitochondrial surface where it interacts with ubiquitin through its ubiquitin binding in ABIN and NEMO (UBAN) domain (Gleason et al., 2011; Heo et al., 2015; Lazarou et al., 2015; Moore and Holzbaur, 2016; Rogov et al., 2014; Wild et al., 2011; Wong and Holzbaur, 2014a; Zhu et al., 2007). Here, OPTN is linked to amyotrophic lateral sclerosis.