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. 2017 Feb 21;18(8):2007–2017. doi: 10.1016/j.celrep.2017.01.079

Figure 6.

Figure 6

Silencing of RNF126 E3 Ligase Promotes Frataxin Accumulation in Cells Derived from FRDA Patients

(A) Fibroblasts derived from two FRDA patients (FA816 and FA078) were transfected with the indicated siRNA. Cell extracts were collected 48 hr post-transfection and analyzed by WB with anti-frataxin antibody, anti-RNF126, and anti-tubulin. Tub, tubulin; mat fxn, mature frataxin.

(B) Graph representing relative mature frataxin abundance as quantitated by densitometric analysis of the blots in (A) and normalized with tubulin levels.

Data represent the mean ± 1 SEM from four independent experiments. The p values were calculated with Student’s t test and were statistically significant (∗p < 0.05; ∗∗p < 0.01) compared to non-targeting siRNA-transfected cells.