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Shang D, Zheng XW, Niiya M, Zheng XL. Apical sorting of ADAMTS13 in vascular endothelial cells and Madin-Darby canine kidney cells depends on the CUB domains and their association with lipid rafts. Blood 2006; 108:2207-2215. The study shows that endothelial cells release and sort ADAMTS13 to the lumen site of cells and the polarity is determined by lipid raft-mediated signals through the CUB domain.
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Luken BM, Kaijen PH, Turenhout EA, et al. Multiple B-cell clones producing antibodies directed to the spacer and disintegrin/thrombospondin type-1 repeat 1 (TSP1) of ADAMTS13 in a patient with acquired thrombotic thrombocytopenic purpura. J Thromb Haemost 2006; 4:2355-2364.
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Luken BM, Turenhout EA, Kaijen PH, et al. Amino acid regions 572-579 and 657-666 of the spacer domain of ADAMTS13 provide a common antigenic core required for binding of antibodies in patients with acquired TTP. Thromb Haemost 2006; 96:295-301. The authors have mapped the core epitopes of ADAMTS13 autoantibodies to two short sequences in the spacer domain.
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ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage
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Soejima K, Matsumoto M, Kokame K, et al. ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage. Blood 2003; 102:3232-3237.
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(2003)
Blood
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Soejima, K.1
Matsumoto, M.2
Kokame, K.3
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50
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28844502248
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Enzymatically active ADAMTS13 variants are not inhibited by anti-ADAMTS13 autoantibodies: A novel therapeutic strategy?
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Zhou W, Dong L, Ginsburg D, et al. Enzymatically active ADAMTS13 variants are not inhibited by anti-ADAMTS13 autoantibodies: a novel therapeutic strategy? J Biol Chem 2005; 280:39934-39941.
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(2005)
J Biol Chem
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, pp. 39934-39941
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Zhou, W.1
Dong, L.2
Ginsburg, D.3
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51
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Inhibitory autoantibodies against ADAMTS-13 in patients with thrombotic thrombocytopenic purpura bind ADAMTS-13 protease and may accelerate its clearance in vivo
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Shelat SG, Smith P, Ai J, Zheng XL. Inhibitory autoantibodies against ADAMTS-13 in patients with thrombotic thrombocytopenic purpura bind ADAMTS-13 protease and may accelerate its clearance in vivo. J Thromb Haemost 2006; 4:1707-1717.
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(2006)
J Thromb Haemost
, vol.4
, pp. 1707-1717
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Shelat, S.G.1
Smith, P.2
Ai, J.3
Zheng, X.L.4
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85117737518
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Ono T, Mimuro J, Madoiwa S, et al. Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure. Blood 2006; 107:528-534. This is the first study to demonstrate ADAMTS13 deficiency in sepsis-induced disseminated intravascular coagulation and to detect proteolytic fragments of the metalloprotease in patients' plasma.
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Ono T, Mimuro J, Madoiwa S, et al. Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure. Blood 2006; 107:528-534. This is the first study to demonstrate ADAMTS13 deficiency in sepsis-induced disseminated intravascular coagulation and to detect proteolytic fragments of the metalloprotease in patients' plasma.
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Nguyen TC, Liu A, Liu L, et al. Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis. Hematologica 2007; 91:121-124. This study links acquired ADAMTS13 deficiency to increased VWF adhesion activity and severe thrombocytopenia in sepsis patients.
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Nguyen TC, Liu A, Liu L, et al. Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis. Hematologica 2007; 91:121-124. This study links acquired ADAMTS13 deficiency to increased VWF adhesion activity and severe thrombocytopenia in sepsis patients.
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Schnog JJ, Hovinga JA, Krieg S, et al. ADAMTS13 activity in sickle cell disease. Am J Hematol 2006; 81:492-498. This is the first study to show that ADAMTS13 deficiency may contribute to vasoocclusion crisis in patients with sickle-cell disease.
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Schnog JJ, Hovinga JA, Krieg S, et al. ADAMTS13 activity in sickle cell disease. Am J Hematol 2006; 81:492-498. This is the first study to show that ADAMTS13 deficiency may contribute to vasoocclusion crisis in patients with sickle-cell disease.
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Platelets treated with ticlopidine are less reactive to unusually large von Willebrand factor multimers than are those treated with aspirin under high shear stress
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Matsumoto M, Kawaguchi S, Ishizashi H, et al. Platelets treated with ticlopidine are less reactive to unusually large von Willebrand factor multimers than are those treated with aspirin under high shear stress. Pathophysiol Haemost Thromb 2005; 34:35-40.
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(2005)
Pathophysiol Haemost Thromb
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, pp. 35-40
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Matsumoto, M.1
Kawaguchi, S.2
Ishizashi, H.3
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High von Willebrand factor levels increase the risk of first ischemic stroke: Influence of ADAMTS13, inflammation, and genetic variability
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Bongers TN, de Maat MP, van Goor ML, et al. High von Willebrand factor levels increase the risk of first ischemic stroke: influence of ADAMTS13, inflammation, and genetic variability. Stroke 2006; 37:2672-2677.
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(2006)
Stroke
, vol.37
, pp. 2672-2677
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Bongers, T.N.1
de Maat, M.P.2
van Goor, M.L.3
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85117738830
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Donadelli R, Orje JN, Capoferri C, et al. Size regulation of von Willebrand factor-mediated platelet thrombi by ADAMTS13 in flowing blood. Blood 2006; 107:1943-1950. This study demonstrates strongly the antithrombtic potential of recombinant ADAMTS13 in vitro.
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Donadelli R, Orje JN, Capoferri C, et al. Size regulation of von Willebrand factor-mediated platelet thrombi by ADAMTS13 in flowing blood. Blood 2006; 107:1943-1950. This study demonstrates strongly the antithrombtic potential of recombinant ADAMTS13 in vitro.
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Chauhan AK, Motto DG, Lamb CB, et al. Systemic antithrombotic effects of ADAMTS13. J Exp Med 2006; 203:767-776. This study shows the formation of ULVWF strings in mouse models and demonstrates the antithrombotic effects of recombinant ADAMTS13.
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Chauhan AK, Motto DG, Lamb CB, et al. Systemic antithrombotic effects of ADAMTS13. J Exp Med 2006; 203:767-776. This study shows the formation of ULVWF strings in mouse models and demonstrates the antithrombotic effects of recombinant ADAMTS13.
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