Article
Oxidized/misfolded superoxide dismutase-1: the cause of all amyotrophic lateral sclerosis?
Annals of neurology - 1 Dec 2007
Kabashi Edor, Valdmanis Paul N, Dion Patrick, Rouleau Guy A
Abstract excerpt
The identification in 1993 of superoxide dismutase-1 (SOD1) mutations as the cause of 10 to 20% of familial amyotrophic lateral sclerosis cases, which represents 1 to 2% of all amyotrophic lateral sclerosis (ALS) cases, prompted a substantial amount of research into the mechanisms of SOD1-mediated toxicity. Recent experiments have demonstrated that oxidation of wild-type SOD1 leads to its misfolding, causing it...
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