“…Decreased ATP production and cellular respiration have been demonstrated clearly in ALS, and after the death of patients with sporadic ALS, decreased electron transport complexes I, II, III, and IV activities were observed in the spinal cord (Song and Pan, 2014;Song et al, 2022). In SOD1 G93A transgenic mice, before the onset of motor symptoms, a reduced mitochondrial respiration rate and impaired ATP synthesis were observed in the spinal cord and brain cord, which persisted throughout the disease process (Sun et al, 2020), accompanied by decreased activities of complex I + III, II + II, I, and IV. As expected, WSJP-treated AR2 mice had an almost normal number of normal mitochondria (Figure 2C) and decreased numbers of abnormal mitochondria and autophagosomes (Figures 2D,E).…”