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Review
. 2009 Oct;34(10):1847-56.
doi: 10.1007/s11064-009-9974-z. Epub 2009 Apr 28.

The human G93A-superoxide dismutase-1 mutation, mitochondrial glutathione and apoptotic cell death

H Muyderman  1 , P G Hutson, D Matusica, M-L Rogers, R A Rush
Affiliations
Review

The human G93A-superoxide dismutase-1 mutation, mitochondrial glutathione and apoptotic cell death

H Muyderman et al. Neurochem Res. 2009 Oct.

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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