SOD1 and amyotrophic lateral sclerosis: Interestingly, a substrate ubiquitination (by STUB1 or maybe other E3-ligases) is not necessarily required for the removal of aberrant proteins by the described BAG3-mediated selective macroautophagy pathway; non-ubiquitinated proteins [e.g., the ALS-linked mutant superoxide dismutase 1 (SOD1G85R)] have been found to be also targeted for autophagic degradation by this particular macroautophagy pathway (Gamerdinger et al., 2011b).