Figure 1.

Lentiviral-mediated short-hairpin RNA (shRNA) sufficiently knocks-down frataxin (FXN). (A) FXN transcript is significantly decreased in shFXN. RNA was reverse transcribed and amplified for FXN and Beta-2-microglobulin (B2M) as a housekeeping control, transcript abundance was quantified using the ΔΔCt method normalized to the empty vector endothelial cells (EVEC). (B) Total protein lysates were run on a bis-tris SDS-PAGE, transferred to nitrocellulose, and probed for FXN and Tata-Binding Protein (TBP) as a housekeeping gene. FXN protein expression is normalized to that of TBP as quantified by densitometry, represented as normalized to pooled EVEC values. Representative blot is shown below. Student’s T-test α = 0.05. * p < 0.05, **** p <0.0001. EVEC and shFXN; n= 23. (B) EVEC; n = 12, and shFXN2; n = 10.