Figure 2
(a, b) Frataxin protein levels in infected fibroblasts. (a) Human; (b) Mouse. Quantification of FXN protein expression after pHR’SIN-cPPT-SFFV-FXN-WPRE LV infection of fibroblasts 2, 8 and 12 weeks post infection. Cells infected with pHR’SIN-cPPT-SFFV-eGFP-WPRE served as controls. Frataxin protein was extracted from fibroblasts and used for a lateral flow immunoassay (‘dipstick’ assay). Frataxin levels of infected human and mouse cells increased as expected to 42- and 17-fold, respectively, compared with untreated FRDA cells 0.48- and 0.2-fold, respectively, of normal fibroblasts. Frataxin protein levels also decreased in both human and mouse fibroblast over time, however, protein levels remained significantly higher than untreated FRDA fibroblasts. Data is represented as the mean of n=2 experiments and samples in triplicate. Frataxin is shown relative to the mean of the normal control is set to 100%. The error bars represent the s.e.m. Significance levels are represented by asterisks: *P<0.05, **P<0.01 and ***P<0.001.
