Article
Mutant SOD1 linked to familial amyotrophic lateral sclerosis, but not wild-type SOD1, induces ER stress in COS7 cells and transgenic mice.
Biochemical and biophysical research communications - 4 Apr 2003
Tobisawa Shinsuke, Hozumi Yasukazu, Arawaka Shigeki, Koyama Shingo, Wada Manabu, Nagai Makiko, Aoki Masashi, Itoyama Yasuto, Goto Kaoru, Kato Takeo
Abstract excerpt
Mutations in a Cu, Zn-superoxide dismutase (SOD1) cause motor neuron death in human familial amyotrophic lateral sclerosis (FALS) and its mouse model, suggesting that mutant SOD1 has a toxic effect on motor neurons. However, the question of how the toxic function is gained has not been answered. Here, we report that the mutant SOD1s linked to FALS, but not wild-type SOD1, aggregated in association with the...
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