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. 2019 May 22;10:2267. doi: 10.1038/s41467-019-09929-w

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© The Author(s) 2019

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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Fig. 4 — Reduced sodium current density is dependent upon reduction of SCN5A expression. a RT-qPCR showing reduced expression of SCN5A gene in CMs generated from K219T-LMNA-iPSCs vs those differentiated from CNTR lines. Data are represented relative to CNTR-CMs and normalized for expression of the housekeeping genes 18S and HGPRT. Data are relative to CMs differentiated from 2 independent lines from 2 CNTR and 3 K219T-LMNA subjects. b Left: Representative Western blot showing reduced Na_v1.5 channel expression in K219T-CMs. Data are representative of one cell line per subject. Right: Na_v1.5 expression quantification in CNTR-CMs and K219T-CMs, calculated as densitometric Na_v1.5 /β-actin ratio (the diagram represents the mean of three independent experiments). c Representative immunofluorescence images for Na_v1.5 (green) and α-sarcomeric actinin (red) in CNTR-CMs and K219T-CMs; nuclei are stained with DAPI (Scale bars: 13 μm). A decrease in the expression of Na_v1.5 in K219T-CMs vs. CNTR-CMs is evident. All values are reported as mean ± SD. *p < 0.05 (unpaired t-test)