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pubmed-article:12221165pubmed:abstractTextTransgenic mice that overexpress a human gene encoding mutant cytosolic superoxide dismutase (SOD1) develop a progressive motor neuron loss that resembles human ALS. Why mutant SOD1 initiates motor neuron death is unknown. One hypothesis proposes that the mutant molecule has enhanced peroxidase activity, reducing hydrogen peroxide (H2O2) to form toxic hydroxyl adducts on critical targets. To test this hypothesis, the authors generated transgenic ALS mice with altered levels of glutathione peroxidase (GSHPx), the major soluble enzyme that detoxifies H2O2.lld:pubmed
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pubmed-article:12221165pubmed:articleTitleSurvival in transgenic ALS mice does not vary with CNS glutathione peroxidase activity.lld:pubmed
pubmed-article:12221165pubmed:affiliationDay Neuromuscular Laboratory, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02115, USA. mcudkowicz@partners.orglld:pubmed
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