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. 2018 May 25;8:8096. doi: 10.1038/s41598-018-25903-w

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© The Author(s) 2018

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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The G553E mutation leads to decreased cell viability and increased caspase-3 activity in HeLa cells and the HD cell model STHdh^Q111 cells. (A,B) The MTT assay was used to measure cell viability in cells expressing the indicated constructs. (A) HeLa cells (ANOVA, p = 0.0037, h = 11.22, n = 6, 2–3 replicates per n). (B) STHdh^Q111 cells (paired t-test, p = 0.0234, n = 5, 3 replicates per n). (C,D) Caspase-3 activity was measured in cells expressing the indicated constructs by following cleavage of the fluorescent substrate Ac-DEVD-AFC. Caspase-3 activity is represented as relative fluorescence units normalised to 17Q-FC. (C) HeLa (ANOVA, p = 0.0218, h = 7.652, n = 5, 3 replicates per n). (D) STHdh^Q111 cells (paired t-test, p = 0.0179, n = 5, 3 replicates per n). (A) and (C) Represent Kruskal-Wallis non-parametric ANOVA with Dunn’s Multiple comparison test post hoc analysis. *p < 0.05, **p < 0.01). (B) and (D) Represent 2-tailed paired Student’s t-test. Error bars represent SEM.