Given that PACER is an enhancer of autophagy, we expected that the gain-of function of PACER at the neuronal level should favor the clearance of polyubiquitinated proteins via autophagy, in addition to the cytotoxic protein aggregates of SOD1 that have not been able to be degraded by the proteasome under ALS conditions. Here, RUBCNL is linked to amyotrophic lateral sclerosis.