Encyclopedia of Metalloproteins

2013 Edition
| Editors: Robert H. Kretsinger, Vladimir N. Uversky, Eugene A. Permyakov

Copper-Zinc Superoxide Dismutase and Lou Gehrig’s Disease

  • Herman Louis Lelie
  • Joan Selverstone Valentine
Reference work entry
DOI: https://doi.org/10.1007/978-1-4614-1533-6_103



Copper-zinc superoxide dismutase (SOD1) is an antioxidant metalloenzyme that is present throughout the body. It catalyzes the conversion of two molecules of superoxide into hydrogen peroxide and molecular oxygen in a reaction that involves alternate oxidation and reduction of the active site copper ion. Amyotrophic lateral sclerosis (ALS, Lou Gehrig’s disease) is a fatal neurodegenerative disease characterized by progressive loss of motor neurons, leading inevitably to paralysis and death, usually within 3–5 years. Onset of disease is usually after age 50, and there is no cure. Most cases have no known genetic component, but in a small fraction of cases, ALS is caused by point mutations in SOD1. This linkage of SOD1 and ALS, first published in 1993, provided the first experimental route to...

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Herman Louis Lelie
    • 1
  • Joan Selverstone Valentine
    • 1
  1. 1.Department of Chemistry and BiochemistryUniversity of CaliforniaLos AngelesUSA