Cell-permeable peptide antioxidants as a novel therapeutic approach in a mouse model of amyotrophic lateral sclerosis

Introduction: Reactive oxygen species (ROS) play a major role in the pathogenesis of neurodegenerative diseases. They are important contributors to necrotic and apoptotic cell death. Structural and functional abnormalities of mitochondria resulting in the generation of ROS, aggregate formation and the release of proapoptotic factors are implicated in ALS pathogenesis. Results: In the present study we investigated a novel peptide antioxidant (SS-31) targeted to the inner mitochondrial membrane for its therapeutic effects both in vitro and in vivo in the G93A mouse model of Amyotrophic Lateral Sclerosis (ALS). SS-…