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. 2018 Jul 23;8:11058. doi: 10.1038/s41598-018-29302-z

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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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Spontaneous activity in myotubes from patient 1. (A) Representative recording of spontaneous APs. When voltage membrane was unstable, firing frequency was not homogeneous either among the different cells and also within a single cell recording. (B) Uprising phase slope/overshoot relationship. Although the characteristics of spontaneous APs lay in an heterogeneous range of values, (dV/dt)_MAX measured during in the phase 0 of an AP could be related to the overshoot of the same AP (n = 14). (C) Variability of APD₃₀, APD₅₀, APD₇₀ and APD₉₀ in spontaneous action potentials (n = 14). Each filled symbol represents a single myotubes, box plots contain data between percentile 25 and 75, the straight line and the white dot sets the median and the mean values respectively.