@article{3076,
  keywords = {Animals, Humans, Signal Transduction, Mutation, Green Fluorescent Proteins, Phenotype, Cells, Cultured, Transfection, Fluorescent Antibody Technique, Mice, Knockout, Actins, Homeodomain Proteins, Tumor Suppressor Proteins, Cell Movement, Protein Multimerization, Genetic Predisposition to Disease, Actin Cytoskeleton, Cadherins, Endothelial Cells, Craniofacial Abnormalities, Endothelium, Lymphatic, Lymphangiectasis, Intestinal, Lymphangiogenesis, Lymphatic Vessels, Lymphedema},
  author = {Francoise Pujol and Tina Hodgson and Ines Martinez-Corral and Anne-Catherine Prats and Danelle Devenport and Masatoshi Takeichi and Elisabeth Genot and Taija M{\"a}kinen and Philippa Francis-West and Barbara Garmy-Susini and Florence Tatin},
  title = {Dachsous1-Fat4 Signaling Controls Endothelial Cell Polarization During Lymphatic Valve Morphogenesis-Brief Report.},
  abstract = { <p><b>OBJECTIVE: </b>The purpose of this study was to investigate the role of Fat4 and Dachsous1 signaling in the lymphatic vasculature.</p><p><b>APPROACH AND RESULTS: </b>Phenotypic analysis of the lymphatic vasculature was performed in mice lacking functional  or . The overall architecture of lymphatic vasculature is unaltered, yet both genes are specifically required for lymphatic valve morphogenesis. Valve endothelial cells (Prox1 [prospero homeobox protein 1] cells) are disoriented and failed to form proper valve leaflets. Using Lifeact-GFP (green fluorescent protein) mice, we revealed that valve endothelial cells display prominent actin polymerization. Finally, we showed the polarized recruitment of Dachsous1 to membrane protrusions and cellular junctions of valve endothelial cells in vivo and in vitro.</p><p><b>CONCLUSIONS: </b>Our data demonstrate that Fat4 and Dachsous1 are critical regulators of valve morphogenesis. This study highlights that valve defects may contribute to lymphedema in Hennekam syndrome caused by Fat4 mutations.</p> },
  year = {2017},
  journal = {Arterioscler Thromb Vasc Biol},
  volume = {37},
  pages = {1732-1735},
  month = {2017 Sep},
  issn = {1524-4636},
  doi = {10.1161/ATVBAHA.117.309818},
  language = {eng},
}