PI3 kinase inhibition improves vascular malformations in mouse models of hereditary haemorrhagic telangiectasia

• Published in: Nature communications Vol. 7; no. 1; pp. 13650 - 12
• Main Authors: Ola, Roxana, Dubrac, Alexandre, Han, Jinah, Zhang, Feng, Fang, Jennifer S, Larrivée, Bruno, Lee, Monica, Urarte, Ana A, Kraehling, Jan R, Genet, Gael, Hirschi, Karen K, Sessa, William C, Canals, Francesc V, Graupera, Mariona, Yan, Minhong, Young, Lawrence H, Oh, Paul S, Eichmann, Anne
• Format: Journal Article
• Language: English
• Published: England Nature Publishing Group 29.11.2016; Nature Portfolio
• Subjects: Activin Receptors, Type I - metabolism; Angiogenesis; Animals; Bone Morphogenetic Proteins - metabolism; Disease Models, Animal; Gene Deletion; Genes; Hemorrhage; Hemorràgia; Human Umbilical Vein Endothelial Cells - metabolism; Humans; Kinases; Ligands; Malalties vasculars; Medicine; Mice; Models, Biological; Mutation; Neovascularization, Pathologic - drug therapy; Oncology; Phosphatidylinositol 3-Kinases - antagonists & inhibitors; Phosphatidylinositol 3-Kinases - metabolism; Physiology; Protein Kinase Inhibitors - pharmacology; Protein Kinase Inhibitors - therapeutic use; Protein kinases; Proteïnes quinases; Rates (Animals de laboratori); Rats as laboratory animals; Retina - pathology; Signal Transduction - drug effects; Telangiectasia, Hereditary Hemorrhagic - complications; Vascular diseases; Vascular endothelial growth factor; Vascular Endothelial Growth Factor Receptor-2 - metabolism; Vascular Malformations - complications; Vascular Malformations - enzymology; United States > US; Connecticut; Spain

Activin receptor-like kinase 1 (ALK1) is an endothelial serine-threonine kinase receptor for bone morphogenetic proteins (BMPs) 9 and 10. Inactivating mutations in the ALK1 gene cause hereditary haemorrhagic telangiectasia type 2 (HHT2), a disabling disease characterized by excessive angiogenesis wi...