Overall, the impact of SOD1 mutations on autophagy is complicated as on the one hand both mRNA and protein levels of the autophagy regulating protein TFEB were decreased in SOD1 mice [316] and mutant SOD1 (aggregates) are able to sequester essential components of the autophagy pathway such as BECN1 [313] and optineurin [317] while on the other hand, an increase of autophagy was found in SOD1-ALS mice [318,319]. Here, OPTN is linked to amyotrophic lateral sclerosis.