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. 2020 Apr 14;52(4):658–671. doi: 10.1038/s12276-020-0414-0

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© The Author(s) 2020

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. 1 — Primary endothelial cell lines were isolated from the human AVM tissue of three separate patients. The “purity” of primary patient-derived AVMECs was tested by Western blot (a) analysis using CD31 as an endothelial marker and αSMA as a mesenchymal marker. AVMECs express levels of CD31 (b) and αSMA (c) that are comparable with those of commercially available normal vascular endothelial cell lines. AVMEC endothelial identity was further confirmed by immunofluorescence that identified positive expression of endothelial lineage markers (CD31 and VE-cadherin). CD31 was also analyzed by Western blot and immunofluorescence, and VE-cadherin was analyzed by immunofluorescence (d) (CD31 and VE-cadherin = green, DAPI (nucleus) = blue). Original magnification ×200. Interestingly, VE-cadherin expression in AVMECs is not observed at the junctions, which is a pattern often noted in endothelial to mesenchymal transition.