Mitochondria are the primary site of ATP production, regulating calcium homeostasis and signaling, and mediating apoptosis. Therefore, mitochondrial structural and functional integrity is critical in governing cellular health. Electron microscopic studies on postmortem tissues of ALS patients identified structural and morphological abnormalities in mitochondria of skeletal muscle, liver, spinal cord neurons, and motor cortex (Tan et al, 2014). In the SOD1 G93A mouse model, changes in mitochondrial structure, number, and mitochondrial fission protein levels have also been documented (Liu et al, 2013). Similar aberrant mitochondria have also been identified in more recent models of disease, C9ORF72, FUS1, and TDP‐43 (Magrane et al, 2014; Lopez‐Gonzalez et al, 2016; Onesto et al, 2016; Uzhachenko et al, 2017). Mitochondria are also found to be fragmented in cell and animal models expressing ALS‐associated mutant SOD1 and TDP43 (Vande Velde et al, 2011; Song et al, 2013; Pickles et al, 2016; Wang et al, 2016).