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. 2020 Jan 31;10:1393. doi: 10.3389/fgene.2019.01393

Figure 5.

Figure 5

Mitochondrial DNA maintenance in mutant RNASEH1 fibroblasts. (A) Western blot analysis of representative components of the mitochondrial OxPhos complexes I-V in control (C1 and C2) and patient (P) fibroblasts grown in either glucose- or galactose-containing medium. GAPDH was used as loading control. (B) Oxygen consumption (I O2) measurements in control (C1 and C2) and patient (P) fibroblasts grown in either glucose- or galactose-containing medium. Values of basal and maximal respiration along with ATP production-dependent, proton leak respiration, and spare capacity are presented. Data are shown as mean ± SD, n = 4, Student’s unpaired two-tail t-test, **p < 0.01, ***p < 0.001. (C) Mitochondrial membrane potential in control (C1 and C2) and patient (P) fibroblasts grown in either glucose- or galactose-containing medium using JC-1 staining. Data are shown as mean ± SD, n = 4, Student’s unpaired two-tail t-test, ***p < 0.001. (D) Growth curves of control (C1 and C2) and patient (P) fibroblasts grown in either glucose or galactose. Cell growth was monitored continuously in an Incucyte cell imager (Essen Bioscience). Data correspond to one of the three independent experiments carried out, and they are shown as the mean of three technical replicates ± SD.