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. 2018 Dec 26;28(10):1594–1607. doi: 10.1093/hmg/ddy448

Figure 7.

Figure 7

The subcellular location and effect of HA-GRP75 and GRP75 on frataxin levels in FRDA patient-derived cells. Skin fibroblasts from FRDA patient with G130V mutation were transduced with lentivirus carrying pHAGE-HA-GRP75 or pHAGE-GRP75 gene for 5 days and subjected to immunofluorescence or western blotting with the indicated antibodies. HA-GRP75 showed diffused staining in the cytosol while GRP75 showed punctate and globular staining and colocalized with mitotracker (A), suggesting the mitochondrial localization. Both HA-GRP75 and GRP75 increased mature frataxin in both typical FRDA patient fibroblasts (pHAGE-GRP75: B and C, n = 4; pHAGE-HA-GRP75: D and E, n = 4) and G130V fibroblasts (pHAGE-GRP75: B and C, n = 4; pHAGE-HA-GRP75: D and E, n = 4). *P < 0.05. Data were shown as mean ± SE. Scale bar = 50 μm.