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. 2019 Mar 28;9:5296. doi: 10.1038/s41598-019-41777-y

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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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Changes of extracellular matrix proteins in cystic airways of type 2 CPAM. (A) Laminin (green) was co-immunostained with vascular endothelial marker PECAM1 (red) and presented in separate panels. (B) Elastin (green) was co-immunostained with smooth muscle cell marker ACTA2 (red). Cell nuclei were counterstained with DAPI (blue). The lower panel shows a magnified small area from upper panel. Arrow: An elastin layer between epithelia and SMCs. Control samples were from normal age-matched lungs, and type 2 CPAM samples (CPAM-1 or -2) were from different patients.