SS-31 was also shown to improve pulmonary hypertension in murine models of the disease (Lu et al., 2015), atherosclerotic plaques in mice in part by increasing SOD activity (Zhang et al., 2017), or oxidative stress in cells from patients with Friedreich ataxia, a progressive neurodegenerative disease (Zhao et al., 2017). This evidence concerns the gene SOD1 and pulmonary arterial hypertension.