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Fig. 3 | Orphanet Journal of Rare Diseases

Fig. 3

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

Fig. 3

Decreased oxygen consumption rates correlate with a loss in OXPHOS complex protein expressions. a Oxygen consumption rate (OCR) of patient and control fibroblasts as measured by micro-oximetry analysis. b Basal and maximal OCR was calculated from repeated experiments (*p < 0.05, ***p < 0.0001; Kruskal-Wallis test with a Dunn's multiple comparisons post test) (c) Western blot analysis of 5 OXPHOS proteins representing each of the complexes involved in oxidative phosphorylation. d 35S-methionine incorporation assay to measure global cytoplasmic translation. Autoradiography represents the 35S signal from newly synthesized proteins and Coomassie represents total protein loading

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172