A low expressor line of transgenic mice carrying a mutant human Cu,Zn superoxide dismutase (SOD1) gene develops pathological changes that most closely resemble those in human amyotrophic lateral sclerosis

@article{Canto1997ALE,
  title={A low expressor line of transgenic mice carrying a mutant human Cu,Zn superoxide dismutase (SOD1) gene develops pathological changes that most closely resemble those in human amyotrophic lateral sclerosis},
  author={Mauro C. Dal Canto and Mark E Gurney},
  journal={Acta Neuropathologica},
  year={1997},
  volume={93},
  pages={537-550}
}
About 15–20% of patients with familial amyotrophic lateral sclerosis (ALS) carry one of several missense mutations in the gene for Cu,Zn superoxide dismutase (SOD1). We have previously reported on an animal model of this disease produced by the transgenic expression of a mutant form of human SOD1 containing a Gly93→Ala amino acid substitution. Several lines of transgenic mice were produced, characterized by a differing tempo and severity of disease that generally correlated with the number of… CONTINUE READING
67 Citations
52 References
Similar Papers

Citations

Publications citing this paper.
Showing 1-10 of 67 extracted citations

References

Publications referenced by this paper.
Showing 1-10 of 52 references

Characterization of intracytoplasmic abnormalities in low expressor SOD 1 transgenic mice with motor neuron disease ( Abstract )

  • LI Bruijn, MW Becher, MK Lee, DW Cleveland, DL Price
  • J Neuropathol Exp Neurol
  • 1996

Mutations of human Cu , Zn superoxide dismutase expressed in transgenic mice cause motor neuron disease

  • M Gurney, A Chiu, MC DalCanto, J Trojanowski, V Lee
  • 1996

Neuropathology of familial ALS with a mutation of the superoxide dismutase 1 gene

  • S Kato, M Shimoda, +4 authors E Ohama
  • 1996

Presence of superoxide dismutase - 1 ( SOD 1 ) immunoreactivity in hyaline inclusion bodies of the spinal cord neurons of transgenic mice for mutant SOD 1 gene

  • N Shibata, A Hirano, +4 authors K Asayama
  • J Neuropathol Exp Neurol
  • 1996

Similar Papers

Loading similar papers…