Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury

  title={Motor neurons in Cu/Zn superoxide dismutase-deficient mice develop normally but exhibit enhanced cell death after axonal injury},
  author={Andrew G. Reaume and Jeffrey L. Elliott and Eric K. Hoffman and Neil W. Kowall and Robert J. Ferrante and Donald R Siwek and Heide M. Wilcox and Dorothy G. Flood and M. Flint Beal and Robert H. Brown and Richard W. Scott and William Douglas Snider},
  journal={Nature Genetics},
The discovery that some cases of familial amyotrophic lateral sclerosis (FALS) are associated with mutations in the gene encoding Cu/Zn superoxide dismutase (SOD1) has focused much attention on the function of SOD1 as related to motor neuron survival. Here we describe the creation and characterization of mice completely deficient for this enzyme. These animals develop normally and show no overt motor deficits by 6 months in age. Histological examination of the spinal cord reveals no signs of… Expand

Paper Mentions

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ALS-Linked Cu/Zn-SOD Mutation Impairs Cerebral Synaptic Glucose and Glutamate Transport and Exacerbates Ischemic Brain Injury
  • Zhihong Guo, M. Kindy, I. Kruman, M. Mattson
  • Biology, Medicine
  • Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism
  • 2000
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