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

Muyderman, H., Hutson, P. G., Matusica, D., Rogers, M. L. and Rush, R. A. (2009) The human G93A-superoxide dismutase-1 mutation, mitochondrial glutathione and apoptotic cell death. Neurochemical Research, 34 10: 1847-1856. doi:10.1007/s11064-009-9974-z


Author Muyderman, H.
Hutson, P. G.
Matusica, D.
Rogers, M. L.
Rush, R. A.
Title The human G93A-superoxide dismutase-1 mutation, mitochondrial glutathione and apoptotic cell death
Journal name Neurochemical Research   Check publisher's open access policy
ISSN 0364-3190
1573-6903
Publication date 2009-10
Sub-type Critical review of research, literature review, critical commentary
DOI 10.1007/s11064-009-9974-z
Volume 34
Issue 10
Start page 1847
End page 1856
Total pages 10
Editor Philip M. Beart
Vladimir J. Balcar
Place of publication New York, NY, United States
Publisher Springer
Language eng
Formatted 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 SOD1G93A mutation, putting forward the hypothesis that this antioxidant pool provides a potentially useful target for therapeutic intervention.
Keyword NSC-34
Amyotrophic lateral sclerosis
Motor neuron
Transgenic
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ
Additional Notes Special Issue in Honor of Dr. Graham A. R. Johnston

Document type: Journal Article
Sub-type: Critical review of research, literature review, critical commentary
Collections: Queensland Brain Institute Publications
ERA 2012 Admin Only
 
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Created: Tue, 18 Oct 2011, 11:39:08 EST by Dusan Matusica on behalf of Queensland Brain Institute