Through an interactome analysis of misfolded SOD1 conformers in mitochondria from the ALS rat model, a recent paper identified the E3 ubiquitin ligase TNF receptor-associated factor 6, a multifunctional protein that plays a role in autophagy and mitophagy modulation [21], as a mutSOD1 interactor. Here, SOD1 is linked to amyotrophic lateral sclerosis.