Fig. 4.

UCM promote frataxin precursor accumulation by preventing K147-dependent degradation.

(A) 293 Flp-In cells stably expressing frataxin^1 -210 (293-frataxin) or the lysine-mutant frataxin^K147R (293-frataxin^K147R) were treated for 24 hrs with 10 μM of the indicated UCM. Proteins were resolved on SDS–PAGE and revealed with anti-frataxin antibody or anti-tubulin, as a loading control. Pre: precursor; tub: tubulin. (B) The graph represents relative frataxin precursor levels as quantified by densitometric analysis. Data represent the mean ± SEM from five different independent experiments. P-values were calculated with Student's t-test and were statistically significant (*P < 0.05; **P < 0.01).