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. 2021 Nov 8;13(12):e14397. doi: 10.15252/emmm.202114397

Figure 4. Lentiviral transduction of patient fibroblasts with wild‐type UQCRH, but not UQCRHL ameliorates the CIII defect.

Figure 4

  1. Western blot analysis of control (n = 2, C1‐2) and patient fibroblasts transduced with a lentiviral vector (pLVX) containing wild‐type UQCRH. Expression of wild‐type UQCRH was induced using various concentrations of doxycycline (dox) up to 20 ng/ml for 72 h.
  2. BN‐PAGE analysis of control (n = 2, C1‐2) and patient fibroblasts transduced with a lentiviral vector (pLVX) containing wild‐type UQCRH. Transduced fibroblasts were either uninduced or induced with 20 ng/ml doxycycline (dox) for 72 h.
  3. Immunofluorescence for UQCRC2 (green), VDAC1/porin (red) and nuclei (DAPI, blue), in control (NHDF) fibroblasts.
  4. Immunofluorescence for UQCRC2 (green), VDAC1/porin (red) and nuclei (DAPI, blue), in patient fibroblasts.
  5. Immunofluorescence for UQCRC2 (green), VDAC1/porin (red) and nuclei (DAPI, blue), in control (NHDF) fibroblasts transduced with wild‐type UQCRH.
  6. Immunofluorescence for UQCRC2 (green), VDAC1/porin (red) and nuclei (DAPI, blue), in patient fibroblasts transduced with wild‐type (WT) UQCRH.
  7. Immunofluorescence for UQCRC2 (green), VDAC1/porin (red) and nuclei (DAPI, blue), in control (NHDF) fibroblasts transduced with the pseudogene UQCRHL.
  8. Immunofluorescence for UQCRC2 (green), VDAC1/porin (red) and nuclei (DAPI, blue), in patient fibroblasts transduced with the pseudogene UQCRHL.
  9. Graph indicating the staining intensity of UQCRC2 in control (NHDF) fibroblasts (green) and patient fibroblasts (red), those respective cell lines transduced (T) with wild‐type UQCRH (diagonal lines) and those cell lines transduced with the pseudogene UQCRHL (vertical lines) measured using Image J, Data are given as mean ± SEM. One‐way ANOVA (Kruskal‐Wallis test), *P < 0.05; n = 3 macroscopic field (10–14 cells for macroscopic field were measured).