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Fig. 2 | Orphanet Journal of Rare Diseases

Fig. 2

From: Impaired activity of CCA-adding enzyme TRNT1 impacts OXPHOS complexes and cellular respiration in SIFD patient-derived fibroblasts

Fig. 2

Mitochondria are not altered in patient-derived fibroblasts. a Electron microscopy images of control and patient-derived fibroblasts at 3000X and 30,000X. The red box identifies the area under 30,000X magnification. b The number of mitochondria was determined using ImageJ software by manual counting from 10 cells each from control or patients from the EM images. c Immunofluorescence images of the mitochondrial membrane potential as monitored by the incorporation of the TMRE dye and compared to the total mitochondrial stain as measured by MitoTracker. Nuclei are identified by Hoechst stain. (bar = 50 μm) (d) The relative TMRE intensity was measured and compared to cells treated with the uncoupler CCCP

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172