Early decrease of redox factor-1 in spinal motor neurons of presymptomatictransgenic mice with a mutant SOD1 gene

Oxidative stress has been proposed to play a pivotal role in pathogenesis o f both sporadic and familial amyotrophic lateral sclerosis (ALS). Expressio n of DNA repair enzyme redox factor-1 (Ref-1) protein was examined in the s pinal cord of transgenic mice with an ALS-linked mutant Cu/Zn superoxide di smutase (SOD1) gene. Immunoblotting and immunocytochemical analyses showed that the most spinal motor neurons lost the immunoreactivity for Ref-1 in t he early presymptomatic stage that preceded significant loss of the neurons . The present result suggests that an early impairment of DNA repair in the spinal motor neurons may account for the mutant SOD1-mediated motor neuron al death in this model. (C) 2001 Elsevier Science BY All rights reserved.