Endoglin prevents vascular malformation by regulating flow-induced cell migration and specification through VEGFR2 signalling

Loss-of-function (LOF) mutations in the endothelial cell (EC)-enriched gene endoglin ( ENG) cause the human disease hereditary haemorrhagic telangiectasia-1, characterized by vascular malformations promoted by vascular endothelial growth factor A (VEGFA). How ENG deficiency alters EC behaviour to tr...

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Veröffentlicht in:	Nature cell biology 2017-06, Vol.19 (6), p.639-652
Hauptverfasser:	Jin, Yi, Muhl, Lars, Burmakin, Mikhail, Wang, Yixin, Duchez, Anne-Claire, Betsholtz, Christer, Arthur, Helen M., Jakobsson, Lars
Format:	Artikel
Sprache:	eng
Schlagworte:	14 14/1 14/19 14/63 38/62 38/77 38/89 45/41 631/136/2060/16 631/136/2091 631/80/84 64/110 64/60 82/29 82/51 82/80 96/106 96/31 96/95 AKT protein Animals Anomalies Arteries Arterioles Arteriovenous malformations Arteriovenous Malformations - genetics Arteriovenous Malformations - metabolism Arteriovenous Malformations - pathology Arteriovenous Malformations - prevention & control Cancer Research Cell adhesion & migration Cell Biology Cell Lineage Cell migration Cell Proliferation Cells, Cultured Clonal deletion Development and progression Developmental Biology Disease Models, Animal Endoglin Endoglin - deficiency Endoglin - genetics Endoglin - metabolism Endothelial Cells - metabolism Endothelial Cells - pathology Genetic Predisposition to Disease Glycoproteins Growth factor receptors Health aspects Humans In vitro methods and tests Kinetics Life Sciences Mice, Knockout Mutation Neovascularization, Pathologic Neovascularization, Physiologic Phenotype Phosphatidylinositol 3-Kinase - metabolism Physiological aspects Prevention Proto-Oncogene Proteins c-akt - metabolism RNA Interference Rodents Signal Transduction Signaling Stem Cells Stress, Mechanical Telangiectasia, Hereditary Hemorrhagic - genetics Telangiectasia, Hereditary Hemorrhagic - metabolism Telangiectasia, Hereditary Hemorrhagic - pathology Telangiectasia, Hereditary Hemorrhagic - prevention & control Tissue Culture Techniques Transfection Vascular endothelial growth factor Vascular Endothelial Growth Factor A - metabolism Vascular Endothelial Growth Factor Receptor-2 - genetics Vascular Endothelial Growth Factor Receptor-2 - metabolism Vascular endothelium
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