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. 2021 Feb 4;16:64. doi: 10.1186/s13023-021-01690-y

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© The Author(s) 2021

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. 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 in a credit line to the data.

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Fig. 2 — Real-time respirometry in fibroblasts of LHON patients. Respiratory chain activity was assessed by Real-Time respirometry (Seahorse measurement) in primary fibroblasts derived from a a healthy control, b a patient carrying the m.3460G>A mutation, and c a patient carrying the m.14484T>C mutation. Cells were incubated in the presence of DMSO or STM 150 µM for 3 h before the measurement (Mean, SEM, n = 6). OCR, oxygen consumption rate. d Extracellular acidification rates (ECAR) of primary fibroblasts (as in panels a–c) after uncoupling respiration with FCCP (Mean, SEM, n = 6)