Fig. 1.
Human WT SOD1 modulates the aggregation of mutant SOD1 in cultured cells. A, B & D. Immunoblots of detergent-insoluble (P2 fraction, upper panels) and detergent-soluble fractions (S1 fraction, lower panels) from HEK293FT cells co-transfected with expression plasmids for WT hSOD1 (or GFP) and mutant SOD1 for 24 (A & B) or 48 hours (D). UT: untransfected cells. hWT: cells transfected with WT hSOD1 construct. Mutant SOD1 constructs noted by A4V, G85R, and G93A notation. C & E. Quantification of the relative aggregation potentials of the studied SOD1 mutants when expressed alone and with WT hSOD1. Bars represent mean ± SEM (n = 3–9). Statistical analysis compares the aggregation of WT hSOD1 to each mutant or mutant co-transfected with WT; *p < 0.05, #p < 0.005, n.s. indicates non-significant differences. F. Quantification of the relative aggregation propensity of WT hSOD1 when co-expressed with G85R hSOD1 mutant for 48 hours. The levels of insoluble mutant SOD1 and WT hSOD1 in cells co-transfected with G85R hSOD1 were significantly different from control WT hSOD1 alone *p < 0.05, #p < 0.005.