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. 2017 Aug 29;7:9840. doi: 10.1038/s41598-017-10320-2

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© The Author(s) 2017

Open Access This 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 license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license 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 license, visit http://creativecommons.org/licenses/by/4.0/.

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A working model of SS-31 efficacy in FRDA patient-derived cells in this study. On the one hand, SS-31 increases FXN expression and available iron in mitochondria, reverses the deficiency of iron-sulphur cluster biogenesis and enhances the ETC of respiratory chain and ATP production. On the other hand, SS-31 reduces the ROS level and increases the capacity for defence against oxidative stress. Altogether, SS-31 improves the mitochondrial function of FRDA patient-derived cells through two pathways and acts as a potential drug for the treatment of FRDA.