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. 2018 May 18;9(1):292–304. doi: 10.1080/19491034.2018.1467722

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© 2018 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Figure 1. — Cytokine level is affected in muscular laminopathies. (A) Levels of TGF β2 in sera of healthy donors (control), patients affected by muscular laminopathies (LMNA) and patients affected by other neuromuscular diseases, including Duchenne Muscular dystrophy, Becker muscular dystrophy and Myotonic Dystrophy (others). (B) Levels of TGF β2, IL6, IL17 and FGF-b in sera of controls and LMNA symptomatic patients. Based on cytokine expression pattern, sera from LMNA symptomatic patients are divided in three subgroups (LMNA1, LMNA2, LMNA3); the percentage of patients in each subgroup out of all examined LMNA patients is reported. (C) Levels of TGF β1, TGF β2, TGF β3 in sera of controls and LMNA patients. (D) Representative image of immunofluorescence detection of TGF β2 (green) in control and LMNA muscle tissue (in this case R190Q/R249Q heterozygous compound mutation in LMNA was determined in the EDMD2 patient). Nuclei are stained with DAPI. Bar: 10µm. (E) Scheme of LMNA mutations detected in laminopathic patients examined in this study. Means ± standard deviation are shown in graphs. Statistically significant differences are indicated by an asterisk (p<0.05), double asterisk (p<0.01) or triple asterisk (p< 0.001).