The adaptor protein Ubiquilin-2 (UBQLN2), mutated in ALS and FTD [59,60] has been shown to interact both with LC3 [97,98] to control conversion of LC3-I to LC3-II [98] and with protein aggregates [99,100], particularly K48- and K63-ubiquitinated proteins in autophagosomes [98] (see details below). This evidence concerns the gene UBQLN2 and amyotrophic lateral sclerosis.