Previous studies have shown that SOD1 G93A expression affects muscle structure and mitochondrial functions leading to subsequent oxidative stress and muscle atrophy (Mahoney et al, 2006; Dobrowolny et al, 2008; de Oliveira et al, 2014; Chen et al, 2015). Electron microscopy (EM) analysis of gastrocnemius muscles from WT, SOD1 G93A, and P110‐treated SOD1 G93A demonstrated that fibers from SOD1 G93A transgenic mice exhibit disorganization, significant changes in mitochondrial morphology and position, and disorganization of the sarcotubular system (Fig 4A). Furthermore, mitochondria were frequently abnormally shaped (P = 0.001), with disturbed cristae, and some had vacuolated structures and their density per area was reduced (P = 0.0001; Fig 4C and D). P110‐treated SOD1 G93A mice showed improved mitochondrial structure (P = 0.004), mitochondrial density as well as sarcotubular system while reducing the levels of altered or damaged mitochondria (P = 0.011; Fig 4A, C and D).