Metal-deficient SOD1 in amyotrophic lateral sclerosis

  title={Metal-deficient SOD1 in amyotrophic lateral sclerosis},
  author={James Hilton and Anthony R White and Peter J. Crouch},
  journal={Journal of Molecular Medicine (Berlin, Germany)},
  pages={481 - 487}
Mutations to the ubiquitous antioxidant enzyme Cu/Zn superoxide dismutase (SOD1) were the first established genetic cause of the fatal, adult-onset neurodegenerative disease amyotrophic lateral sclerosis (ALS). It is widely accepted that these mutations do not cause ALS via a loss of antioxidant function, but elucidating the alternate toxic gain of function has proven to be elusive. Under physiological conditions, SOD1 binds one copper ion and one zinc ion per monomer to form a highly stable… 

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