11 Folding and Aggregation of Cu , Zn-Superoxide Dismutase

  title={11 Folding and Aggregation of Cu , Zn-Superoxide Dismutase},
  author={Helen R. Broom and Heather A. Primmer and Jessica A. O. Rumfeldt and Peter B Stathopulos and Kenrick A. Vassall and Y T Hwang and Elizabeth M. Meiering},
1.1 ALS and SOD1 In 1993, a genetic link was established between amyotrophic lateral sclerosis (ALS) and mutant forms of Cu,Zn superoxide dismutase (SOD1) (Deng et al. 1993; Rosen et al. 1993), an antioxidant enzyme that catalyzes the dismutation of the damaging free radical superoxide anion (O2-) to hydrogen peroxide (H2O2) and diatomic oxygen (O2) via cyclic reduction and oxidation of a protein-bound Cu ion (Valentine et al. 2005). Today, over 150, predominantly missense mutations have been… CONTINUE READING
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