Oxidation versus aggregation — how do SOD1 mutants cause ALS?

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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