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Figure S1.The adjusted mean cellular fluorescence of the transfected cells expressing the EGFP fusion of SOD1WT, SOD1A4V, and SOD1A4V variants containing a second mutation. The values were calculated by dividing the mean cellular fluorescence of each sample by the relative expression level to that of SOD1WT. Values = mean ± standard deviation (n = 3). In order to determine whether the mean cellular fluorescence of the SOD1 double mutants is greater than that of SOD1A4V, two-sided Student's t-tests were applied to the data (*P < 0.05; **P < 0.01).

Figure S2.The adjusted mean cellular fluorescence of the transfected cells expressing the EGFP fusion of SOD1WT, SOD1A4V, SOD1A4V/F20A/C111S, and SOD1A4V/F20G/C111S. The values were calculated by dividing the mean cellular fluorescence of each sample by the relative expression level to that of SOD1WT. Values = mean ± standard deviation (n = 3). In order to determine whether the mean cellular fluorescence of the SOD1 triple mutants is greater than that of SOD1A4V, two-sided Student's t-tests were applied to the data (*P < 0.05; NS, not significant). The mean cellular fluorescence of the SOD1A4V/F20A/C111S is not significantly different from that of SOD1WT.

Table SI. Mean cellular fluorescence intensities.

Table SII. The percentage of transfected HEK293T cells exhibiting SOD1 aggregates.

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