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  • Title: The human G93A-superoxide dismutase-1 mutation, mitochondrial glutathione and apoptotic cell death.
    Author: Muyderman H, Hutson PG, Matusica D, Rogers ML, Rush RA.
    Journal: Neurochem Res; 2009 Oct; 34(10):1847-56. PubMed ID: 19399611.
    Abstract:
    Mutations in Cu/Zn superoxide dismutase are a cause of motor neuron death in about 20% of cases of familial amyotrophic lateral sclerosis (ALS). Although the molecular mechanism of which these mutations induce motor neuron cell death is to a large extent unknown, there is significant evidence that effects on mitochondrial function and development of oxidative stress make a major contribution to the selective death of motor neurons in this disease. In this overview article we review the current understanding of mutant SOD1-mediated motor neuron degeneration in ALS with focus on oxidative damage and mitochondrial dysfunction. We also present novel information on the role of mitochondrial glutathione for the survival of NSC-34 cells stably transfected with the human SOD1(G93A) mutation, putting forward the hypothesis that this antioxidant pool provides a potentially useful target for therapeutic intervention.
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