Characterization of lymphatic malformations using primary cells and tissue transcriptomes.

Autor: Kaipainen A; Vascular Biology Program, Department of Surgery, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA., Chen E; Vascular Biology Program, Department of Surgery, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA., Chang L; Vascular Biology Program, Department of Surgery, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA., Zhao B; Department of Ophthalmology and Visual Sciences, University of Kentucky, Lexington, KY, USA., Shin H; Department of Biomedical Engineering, University of Kentucky, Lexington, KY, USA., Stahl A; Vascular Biology Program, Department of Surgery, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA., Fishman SJ; Department of Surgery, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA., Mulliken JB; Department of Plastic and Oral Surgery, Department of Surgery, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA., Folkman J; Vascular Biology Program, Department of Surgery, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA., Huang S; Vascular Biology Program, Department of Surgery, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA., Fannon M; Vascular Biology Program, Department of Surgery, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA.; Department of Ophthalmology and Visual Sciences, University of Kentucky, Lexington, KY, USA.
Jazyk: angličtina
Zdroj: Scandinavian journal of immunology [Scand J Immunol] 2019 Oct; Vol. 90 (4), pp. e12800. Date of Electronic Publication: 2019 Aug 01.
DOI: 10.1111/sji.12800
Abstrakt: Lymphatic malformations (LMs) are disfiguring congenital anomalies characterized by aberrant growth of lymphatic vessels. They are broadly categorized histopathologically as macrocystic and microcystic. Although sclerotherapy has shown some success in the treatment of macrocystic malformations, there has been less progress with developing treatment strategies for microcystic malformations. In this study, we characterized lymphatic endothelial cells isolated from lymphatic and lymphaticovenous malformations. When compared to cells from normal lymphatic vessels, we found that the primary cultured malformed cells are morphologically different and also exhibited differences in binding, proliferation, migration and tube formation. Transcriptome analysis identified several genes whose expression was substantially higher in malformed compared to normal lymphatic endothelium, including DIRAS3 and FOXF1. Further analysis of LM tissue samples revealed distinguishing gene expression patterns that could pave the way to understanding the molecular pathogenesis of LMs. Based on gene expression signatures, we propose a new hypothesis that the subtype of localized LMs could be formed because of disruptions in lymph node development.
(© 2019 The Foundation for the Scandinavian Journal of Immunology.)
Databáze: MEDLINE
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