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Rescue of fALS-variant G85R SOD1 activity by chemical cross-linking (see Fig. S6). G85R is a metal-deficient and inactive variant. In the presence of excess copper, zinc, or BMOE G85R activity increases. Most notably, G85R in the presence of copper, zinc, and DTME confers WT-like levels of activity. Thus, the increase in stability of G85R also corresponds to an increase in activity (Fig. S6). As expected WT SOD1 and G93A SOD1, a fully active mutant, appear to be unaffected.
