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Titolo:
Anti-endothelial cell antibodies from patients with thrombotic thrombocytopenic purpura specifically activate small vessel endothelial cells
Autore:
Praprotnik, S; Blank, M; Levy, Y; Tavor, S; Boffa, MC; Weksler, B; Eldor, A; Shoenfeld, Y;
Indirizzi:
Chaim Sheba Med Ctr, Autoimmune Dis Res Unit, IL-52621 Tel Hashomer, Israel Chaim Sheba Med Ctr Tel Hashomer Israel IL-52621 21 Tel Hashomer, Israel Chaim Sheba Med Ctr, Dept Med B, IL-52621 Tel Hashomer, Israel Chaim ShebaMed Ctr Tel Hashomer Israel IL-52621 21 Tel Hashomer, Israel Ctr Clin, Dept Rheumatol, Ljubljana 1000, Slovenia Ctr Clin Ljubljana Slovenia 1000 ept Rheumatol, Ljubljana 1000, Slovenia Sorasky Med Ctr, Inst Hematol, IL-54145 Tel Aviv, Israel Sorasky Med Ctr Tel Aviv Israel IL-54145 atol, IL-54145 Tel Aviv, Israel Hop St Louis, INSERM U353, F-75475 Paris, France Hop St Louis Paris France F-75475 is, INSERM U353, F-75475 Paris, France Cornell Univ, Weill Med Coll, New York, NY 10021 USA Cornell Univ New York NY USA 10021 Weill Med Coll, New York, NY 10021 USA
Titolo Testata:
INTERNATIONAL IMMUNOLOGY
fascicolo: 2, volume: 13, anno: 2001,
pagine: 203 - 210
SICI:
0953-8178(200102)13:2<203:ACAFPW>2.0.ZU;2-K
Fonte:
ISI
Lingua:
ENG
Soggetto:
ADHESION MOLECULE EXPRESSION; DISSEMINATED INTRAVASCULAR COAGULATION; HEMOLYTIC-UREMIC SYNDROME; IN-VITRO; ANTIPHOSPHOLIPID ANTIBODIES; PLASMA THROMBOMODULIN; VONWILLEBRAND-FACTOR; PATHOGENIC ROLE; E-SELECTIN; VASCULITIS;
Keywords:
adhesion molecules; anti-endothelial cell antibodies; autoimmunity; endothelial cell; thrombomodulin; thrombotic thrombocytopenic purpura;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
52
Recensione:
Indirizzi per estratti:
Indirizzo: Shoenfeld, Y Chaim Sheba Med Ctr, Autoimmune Dis Res Unit, IL-52621 Tel Hashomer, Israel Chaim Sheba Med Ctr Tel Hashomer Israel IL-52621 er, Israel
Citazione:
S. Praprotnik et al., "Anti-endothelial cell antibodies from patients with thrombotic thrombocytopenic purpura specifically activate small vessel endothelial cells", INT IMMUNOL, 13(2), 2001, pp. 203-210

Abstract

Thrombotic thrombocytopenic purpura (TTP) is an uncommon disease of an unknown etiology, characterized by consumptive thrombocytopenia, microangiopathic hemolytic anemia, fever and acute thrombotic complications, especially within the cerebral circulation. Although anti-endothelial cell antibodies (AECA) have occasionally been shown to be present in TTP, their role in thepathogenesis of the disease has never been ascertained. In the current study we demonstrated the pathogenic activity of affinity-purified anti-endothelial cell F(ab)(2) antibodies (AECA/TTP) from four consecutive patients with active TTP, These AECA/TTP bound to and activated only microvascular endothelial cells (EC) and not large vessel EC, The specificity of AECA/TTP binding to microvascular EC was confirmed by competition assay employing membranes derived from small and large vessels EC, Activation included enhancedIL-6 and von Willebrand factor release from the EC followed by increased expression of adhesion molecules P-selectin, E-selectin and vascular cell adhesion molecule-1 on the EC, as evaluated by ELISA, Increased expression ofadhesion molecules was followed by an increase in monocyte adhesion to EC,The level of soluble thrombomodulin (TM) also increased in the culture medium of activated microvascular EC upon exposure to AECA/TTP antibodies and was directly correlated to a decrease in cell-associated TM. Our data suggest that AECA/TTP directed against microvascular EC could play a pathogenic role in the development of endothelial injury in TTP that leads to thrombosis.

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Documento generato il 21/09/20 alle ore 12:48:44