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. 2016 Sep 28;53(12):846–849. doi: 10.1136/jmedgenet-2016-104194

Figure 2.

Figure 2

Western blot (WB) immunodetection and enzymatic activities. (A) WB immunodetection of SDS-PAGE in primary fibroblasts. P, patient, C1 and C2, control cell lines. (B) WB immunodetection of BNGE in immortalised patient (Pi) and control (C3i) fibroblasts. Immunovisualised subunits are between brackets. (C) WB immunodetection of SDS-PAGE in immortalised patient fibroblasts (Pi) and a control (C4i). n.t., not transduced; mock, transduced with empty vector; COA7, transduced with COA7. (D) WB immunodetection of BNGE in COA7-transduced immortalised fibroblasts from patient (Pi) and a control (C4i). (E) Cytochrome c oxidase (COX)/citrate synthase (CS) activity in immortalised fibroblasts of two controls (C3i, C4i), and the patient (Pi). Vertical bars indicate SEM. Statistical analysis was by ANOVA (*p<0.05; **p<0.01; ***p<0.001). (F) WB immunodetection of SDS-PAGE of mitochondria and mitoplasts from HEK293T cells. (G) WB immunodetection of SDS-PAGE of soluble and membrane fractions from intact mitochondria. ANOVA, analysis of variance.