Stabilization of mutant Cu/Zn superoxide dismutase (SOD1) protein by coexpressed wild SOD1 protein accelerates the disease progression in familial amyotrophic lateral sclerosis mice.

@article{Fukada2001StabilizationOM,
  title={Stabilization of mutant Cu/Zn superoxide dismutase (SOD1) protein by coexpressed wild SOD1 protein accelerates the disease progression in familial amyotrophic lateral sclerosis mice.},
  author={Kei Fukada and Seiichi Nagano and Mamoru Satoh and Chiharu Tohyama and Tohru Nakanishi and Akira Shimizu and Takehiko Yanagihara and Saburo Sakoda},
  journal={The European journal of neuroscience},
  year={2001},
  volume={14 12},
  pages={2032-6}
}
Transgenic mice carrying familial amyotrophic lateral sclerosis (FALS)-linked mutant Cu/Zn superoxide dismutase (SOD1) genes such as G93A (G93A-mice) and G85R (G85R-mice) genes develop limb paresis. Introduction of human wild type SOD1 (hWT-SOD1) gene, which does not cause motor impairment by itself, into different FALS mice resulted in different effects on their clinical courses, from no effect in G85R-mice to acceleration of disease progression in G93A-mice. However, the molecular mechanism… CONTINUE READING