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. 2017 Jul 18;7:5704. doi: 10.1038/s41598-017-05571-y

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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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Mitochondrial membrane potential analysis. The mitochondrial membrane potential (ΔΨm) was measured in three mutant and three control cell lines via the JC-10 assay. The ratio of the Ex/Em = 488/590 nm and 488/525 nm fluorescence intensities (FL590/FL525) was recorded to determine the ΔΨm level of each sample. The relative ratios of the FL590/FL525 geometric means between the mutant and control cell lines were calculated to reflect the level of ΔΨm. Flow cytometry images of III-12 and III-11 with (a,b) and without (d,e) FCCP. Relative ratio of JC-10 fluorescence intensities at Ex/Em = 490/525 and 490/590 nm in absence (c) and presence (f) of 10 mM of FCCP. The average of three to five determinations for each cell line is shown. Graph details and symbols are explained in the legend to Fig. 2.