Respiration and ROS production in brain and spinal cord mitochondria of transgenic rats with mutant G93a Cu/Zn-superoxide dismutase gene

► The yields of BM and SCM from tgmSOD1 rats were diminished by 27% and 58% respectively. ► State 3 and State 3U respiration rates in tgBM and tgSCM were normal. ► State 4 respiration in tgSCM was increased significantly. ► ROS production with physiologically relevant substrates was increased several fold. ► tgSCM produced two times more ROS than tgBM.