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. 2023 May 25;13:8146. doi: 10.1038/s41598-023-34445-9

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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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SBMA disease-specific hiPSCs efficiently differentiated into mature skeletal muscle and exhibited reduced ACTN3 expression, consistent with the decrease in fast-twitch muscle in SBMA patients. (a) ICC analysis of MyoG (red) and MHC (green) in skeletal muscles derived from SBMA disease-specific hiPSCs and control hiPSCs. SBMA disease-specific hiPSCs could differentiate into mature skeletal muscles as efficiently as control hiPSCs. Scale bar, 200 μm. (b) Quantitative analysis of the parameters of skeletal muscle differentiation (MyoG⁺ cells and MHC⁺ nuclei) and maturation (thickness of myotubes). The data are presented as the mean ± SEM, n = 3. (c,d) Quantitative RT‒PCR analysis of SBMA and control hiPSC-derived skeletal muscles for MYF6, endo-MYOD1, MYOG, MYH2, and TMEM8C. (c) and ACTN3 (d) expressed in fast-twitch muscles. The data are presented as the mean ± SEM, n = 9 (n = 3 each from 3 patients and 3 controls). *p < 0.05, **p < 0.01.