FIG. 9.

Overexpression of FUS protects against rotenone induced cytotoxicity. NSC-34 cells were transfected with wild-type or mutant FUS expression vector followed by treatment with rotenone for 24 h. Trypan blue exclusion assay was performed to detect the dead cells (A). Cultured primary skin fibroblast cells that were obtained from fALS patients or from normal subjects were treated with the indicated concentration of rotenone and the Trypan blue exclusion assay was performed 24 h after treatment (B). Both live and dead cells were counted, and the ratio of dead to total cells was calculated and expressed as fold changes. H2DCFDA and DCFDA fluorescence was measured in NEC-34 cells (C) and skin fibroblast (D) after treatment with rotenone. Cells were also treated with polyethylene glycol-SOD 1 h before rotenone treatment. Each data point represents mean±SD of three to four samples and significant difference compared to respective control is indicated by *p<0.05 and **p<0.01; different from treatment #p<0.05.