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. 2017 Sep 15;5:72. doi: 10.1186/s40478-017-0474-0

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© The Author(s). 2017

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided 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 Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Fig. 1 — Passive sarcomere length-tension relationships of isolated normal and myopathic skinned myofibers. (a) Schematic of experimental setup. (b) Mechanical measurement protocol. Sarcomere length (SL) was increased stepwise from 1.8 to 3.0 μm. (c) Mean SL-dependent passive tension (PT) of normal (CTRL; N = 20 fibers) and MFM-filaminopathy (‘FLN-C’; N = 15 fibers) vastus lateralis muscle fibers, from two different individuals/patients per group. (d) Mean PT of normal (CTRL; N = 14 fibers) and LGMD2A (N = 11 fibers) gastrocnemius muscle fibers, from two different individuals/patients per group. Mean data points were fit with simple exponential functions. Symbols and error bars are means ± SEM; *p < 0.05 in Student’s t-test