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. 2021 Aug 23;29(12):1789–1795. doi: 10.1038/s41431-021-00947-1

Fig. 3. Complementation assay of SLIRP in patient fibroblasts.

Fig. 3

A Mitochondrial transcript steady-state levels assessed by qPT-PCR in patient fibroblasts (denoted as P) and controls (denoted as C). hTBP was used as endogenous control. Data are represented as means ± SD (n = 3). Error bars indicate SD. Student’s t test was used for statistics. *p < 0.05, **p < 0.01, ***p < 0.001. B Transduction of patient fibroblasts with wild-type SLIRP recovers mitochondrial gene expression in galactose medium culture. Patient fibroblasts were stably transduced with lentiviral clones containing wild-type SLIRP (denoted as W), SLIRP containing 5 bp deletion in exon 3 (denoted as D) and SLIRP containing 106 bp intron 1 retention (denoted as I). hTBP was used as endogenous control. Relative RNA expressions of W, D and I were normalized to P. The relative RNA expression data are represented as means ± SD (n = 3). Error bars indicate SD. Student’s t test was used for statistics. *p < 0.05, **p < 0.01, ***p < 0.001. C Transduction of patient fibroblasts with wild-type SLIRP restored complex I and IV activity. The enzyme measurement was performed once because of the extremely early senescence of the transfected cells, which prohibited culturing to sufficiently large numbers of cells. D Transduction of patient fibroblasts with wild-type SLIRP partially restored MT-CO1 protein levels.