These include mutations in the E3 ligase PARKIN and the deubiquitinating enzyme UCHL1, which are both associated with PD [16,17], and mutations in the autophagy receptor SQSTM1 (p62) and UBQLN2 (which has been implicated in both the UPS and autophagy), which are associated with ALS [18,19]. Here, SQSTM1 is linked to amyotrophic lateral sclerosis.