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Oxidation versus aggregation — how do SOD1 mutants cause ALS?

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Multiple models have been proposed for the mechanism by which mutant superoxide dismutase 1 induces motor neuron death and amyotrophic lateral sclerosis. A combination of cell biology and genetics may soon lead to an answer.

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Figure 1: Proposed models for SOD1-mediated toxicity linked to altered conformation and/or aggregation of mutant SOD1 subunits.

Bob Crimi

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Authors and Affiliations

  1. Ludwig Institute for Cancer Research and Departments of Medicine and Neuroscience University of California at San Diego, 9500 Gilman Drive, La Jolla, 92093, California, USA

    Don W. Cleveland & Jian Liu

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  1. Don W. Cleveland
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  2. Jian Liu
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Cleveland, D., Liu, J. Oxidation versus aggregation — how do SOD1 mutants cause ALS?. Nat Med 6, 1320–1321 (2000). https://doi.org/10.1038/82122

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