Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis.

  title={Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis.},
  author={Joan Selverstone Valentine and Peter Doucette and Soshanna Zittin Potter},
  journal={Annual review of biochemistry},
Copper-zinc superoxide dismutase (CuZnSOD, SOD1 protein) is an abundant copper- and zinc-containing protein that is present in the cytosol, nucleus, peroxisomes, and mitochondrial intermembrane space of human cells. Its primary function is to act as an antioxidant enzyme, lowering the steady-state concentration of superoxide, but when mutated, it can also cause disease. Over 100 different mutations have been identified in the sod1 genes of patients diagnosed with the familial form of… CONTINUE READING
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