Figure 3 | Gene Therapy

Figure 3

From: Lentivirus-meditated frataxin gene delivery reverses genome instability in Friedreich ataxia patient and mouse model fibroblasts

Figure 3

Localization of intermediate and mature forms of frataxin to subcellular compartments. Western analysis of intermediate and mature forms of the frataxin protein following cell fractionation was performed on normal, FRDA and LV GFP and LV FXN-treated human (a) and mouse fibroblasts (b). Frataxin isoforms were identified using antibodies that recognize the 19 kDa intermediate and 14 kDa mature forms of the protein (shown by arrows). Controls staining for the mitochondrial fraction used SOD2, for the cytoplasmic fraction used GAPDH and for the nuclear fraction used EMERIN. Only in the mouse could mature frataxin be identified in the nuclear fraction (shown by *).

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