Mutations in the autophagy adaptors optineurin (OPTN), ubiquilin 2 and p62 (SQSTM1) found in ALS point to defects in the aggrephagy pathway, a cargo-specific form of autophagy, and suggest defects in protein homeostasis may contribute significantly to disease pathogenesis [31]. Here, OPTN is linked to amyotrophic lateral sclerosis.