title: Endothelial cells: source, barrier, and target of defensive mediators.
abstract: Endothelium is strategically located at the interface between blood and interstitial tissues, placing thus endothelial cell as a key player in vascular homeostasis. Endothelial cells are in a dynamic equilibrium with their environment and constitute concomitantly a source, a barrier, and a target of defensive mediators. This review will discuss the recent advances in our understanding of the complex crosstalk between the endothelium, the complement system and the hemostasis in health and in disease. The first part will provide a general introduction on endothelial cells heterogeneity and on the physiologic role of the complement and hemostatic systems. The second part will analyze the interplay between complement, hemostasis and endothelial cells in physiological conditions and their alterations in diseases. Particular focus will be made on the prototypes of thrombotic microangiopathic disorders, resulting from complement or hemostasis dysregulation-mediated endothelial damage: atypical hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Novel aspects of the pathophysiology of the thrombotic microangiopathies will be discussed.
authors: INSERM UMRS 1138, Cordeliers Research Center, Université Pierre et Marie Curie (UPMC-Paris-6) and Université Paris Descartes Sorbonne Paris-Cité, Paris, France. lubka.roumenina@crc.jussieu.fr.
authors: Lubka T
authors: LT
authors: Roumenina
authors: Institute of Cardiovascular Sciences, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.
authors: Julie
authors: J
authors: Rayes
authors: INSERM UMR 995, Lille, France.|Nephrology Department, CHU Lille, Lille, France.
authors: Marie
authors: M
authors: Frimat
authors: INSERM UMRS 1138, Cordeliers Research Center, Université Pierre et Marie Curie (UPMC-Paris-6) and Université Paris Descartes Sorbonne Paris-Cité, Paris, France.|Assistance Publique - Hôpitaux de Paris, Service d'Immunologie Biologique, Hôpital Européen Georges Pompidou, Paris, France.
authors: Veronique
authors: V
authors: Fremeaux-Bacchi
mesh_terms: D000818:Animals; D065766:Atypical Hemolytic Uremic Syndrome / Q000276:immunology*; D001777:Blood Coagulation; D003165:Complement System Proteins / Q000378:metabolism*; D042783:Endothelial Cells / Q000502:physiology*; D006487:Hemostasis; D006706:Homeostasis; D006801:Humans; D011697:Purpura, Thrombotic Thrombocytopenic / Q000276:immunology*; D057049:Thrombotic Microangiopathies / Q000276:immunology*
chemical_list: D003165:Complement System Proteins
keywords: atypical hemolytic uremic syndrome; complement system; endothelial cells; hemolysis; hemostasis; thrombosis