Mutations in the ubiquitin binding domain of OPTN, which were identified by targeted sequencing in Japanese patients with ALS (Maruyama et al., 2010), fail to rescue recruitment of the autophagosomal light chain 3 (LC3; MAP1LC3A/B/C) protein and mitophagy in OPTN-depleted cultured cells (Wong and Holzbaur, 2014). Here, MAP1LC3A is linked to amyotrophic lateral sclerosis.