Fig. 3.

Polyamide FA1 increases the levels of frataxin mRNA and protein in an FRDA lymphoid cell line. (A) Measurement of frataxin mRNA levels, relative to that of GAPDH, in cell lines derived from an unaffected individual (GM15851) and an FRDA patient (GM15850) by qRT-PCR (44). Polyamides FA1 and FA2 were included in the cell culture medium at the indicated concentrations, and frataxin and GAPDH mRNA were determined after 7 days, with media and polyamide replenished on days 3 and 5. Error bars are derived from the percent error of the average and standard deviation of the change in cycle threshold between frataxin and GAPDH for triplicate experiments, with triplicate qRT-PCR determinations for each experiment. (B) Effects of polyamides on frataxin protein in cultured lymphoid cells. FRDA or control cells were incubated as in A before Western blot analysis with antibody to human frataxin or actin. Equivalent amounts of total-cell-extract protein were loaded in each lane.