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. 2016 Jun 18;11:79. doi: 10.1186/s13023-016-0466-3

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© The Author(s). 2016

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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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