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. 2023 May 10;56:23. doi: 10.1186/s40659-023-00437-2

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© The Author(s) 2023

Open AccessThis 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/. 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 in a credit line to the data.

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Fig. 1 — General phenotypic characterization of RDEB-CW cells. (a) Western blot of protein lysates derived from fibroblast cultures confirms the C7 deficiency in all samples coming from RDEB patients used in this study, with its respective densitometric analysis in (b). (c) Representative IIF images showing molecular markers for fibroblasts (FAP, vimentin) and white blood cells (CD45). Nuclei were co-stained with DAPI. Bar: 100 μm. (d-f) Representative bright field images of NHFs, RDEBs, RDEB-CWs and RDEB-AWs fibroblast cultures used for morphological evaluation. Black arrows indicate cell protrusions. The morphological parameters analyzed were cell area (e) and the mean protrusions per cell (f). For the quantitative analysis in (e-f), a total of 75 cells were used for each condition (225 cells per experimental group). Bar: 100 μm. All data are expressed as mean ± SD. All these experiments were performed with n = 3–4 per condition. Asterisks indicate significant differences by one-way ANOVA with Tukey post-hoc (p < 0.05)