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. 2023 Jun 26;13:10374. doi: 10.1038/s41598-023-37496-0

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© The Author(s) 2023

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 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/.

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Effect of AR42 on gem numbers in type II SMA fibroblasts. GM03813 type II SMA fibroblasts were treated with differing doses of AR42, AR19, 4PBA or TSA for 5 days (n = 3/dose/drug). The number of SMN-positive gems within 100 randomly selected nuclei was counted. Representative images are shown for GM03813 SMA fibroblasts treated with 1 μM AR42 (A), 1 μM AR19 (B), 1 μM 4PBA (C), 100 nM TSA (D) or DMSO (E) as well as for GM03814 carrier fibroblasts (F). The scale bars (yellow lines) represent 10 μm. The gem count analysis was expressed as (G) the number of gems per 100 nuclei, (H) the proportion of cells containing gems and (I) the proportion of cells containing multiple gems. The data are presented as mean ± standard error. The asterisk (*) denotes a statistically significant (p ≤ 0.05; one-way ANOVA with Bonferroni post hoc test) difference between drug- and vehicle-treated GM03813 SMA cells.