In our study, we found that the levels of ETC complex subunits, including NDUFA9, SDHA, Cyt b, COX I and ATP5A, as well as the SOD activity and the GSH/GSSG ratio, were obviously decreased in PHG patients and mice with PVL but could be restored by Nec-1 or Mdivi-1 treatment in mice with PVL. Here, SOD1 is linked to periventricular leukomalacia.