SEARCH:   Advanced

Prepublished online as a Blood First Edition Paper on July 25, 2002; DOI 10.1182/blood-2002-05-1401. This Article Full Text Full Text (PDF) All Versions of this Article: 2002-05-1401v1 100/12/4033    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Dong, J.-f. Articles by López, J. A. Search for Related Content PubMed PubMed Citation Articles by Dong, J.-f. Articles by López, J. A. Related Collections Hemostasis, Thrombosis, and Vascular Biology Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 1 December 2002, Vol. 100, No. 12, pp. 4033-4039 HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions Jing-fei Dong, Joel L. Moake, Leticia Nolasco, Aubrey Bernardo, Wendy Arceneaux, Corie N. Shrimpton, Alicia J. Schade, Larry V. McIntire, Kazuo Fujikawa, and José A. López From the Thrombosis Research Section, Department of Medicine, and Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX; Department of Biochemistry, University of Washington, Seattle; and the Cox Laboratory for Bioengineering, Rice University, Houston, TX. Thrombotic thrombocytopenic purpura (TTP) is a devastating thrombotic disorder caused by widespread microvascular thrombi composed of platelets and von Willebrand factor (VWF). The disorder is associated with a deficiency of the VWF-cleaving metalloprotease, ADAMTS-13, with consequent accumulation of ultralarge (UL) VWF multimers in the plasma. ULVWF multimers, unlike plasma forms of VWF, attach spontaneously to platelet GP Ib, a component of the GP Ib-IX-V complex. We have found that ULVWF multimers secreted from stimulated endothelial cells (ECs) remained anchored to the endothelial surface where platelets and Chinese hamster ovary cells expressing the GP Ib-IX-V complex attached to form long beads-on-a-string structures in the presence of fluid shear stresses in both the venous (2.5 dyne/cm2) and arterial (20 and 50 dyne/cm2) ranges. Although measurement of the activity of the ADAMTS-13 VWF-cleaving metalloprotease in vitro requires prolonged incubation of the enzyme with VWF under nonphysiologic conditions, EC-derived ULVWF strings with attached platelets were cleaved within seconds to minutes in the presence of normal plasma (containing approximately 100% ADAMTS-13 activity) or in the presence of partially purified ADAMTS-13. By contrast, the strings persisted for the entire period of perfusion (10 minutes) in the presence of plasma from patients with TTP containing 0% to 10% ADAMTS-13 activity. These results suggest that cleavage of EC-derived ULVWF multimers by ADAMTS-13 is a rapid physiologic process that occurs on endothelial cell surfaces. © 2002 by The American Society of Hematology.   CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: D. D. Wagner and P. S. Frenette The vessel wall and its interactions Blood, June 1, 2008; 111(11): 5271 - 5281. [Abstract] [Full Text] [PDF] B. Hohenstein, A. Braun, K. U. Amann, R. J. Johnson, and C. P. M. Hugo A murine model of site-specific renal microvascular endothelial injury and thrombotic microangiopathy Nephrol. Dial. Transplant., April 1, 2008; 23(4): 1144 - 1156. [Abstract] [Full Text] [PDF] B.-J. H. van den Born, N. V. van der Hoeven, E. Groot, P. J. Lenting, J. C.M. Meijers, M. Levi, and G. A. van Montfrans Association Between Thrombotic Microangiopathy and Reduced ADAMTS13 Activity in Malignant Hypertension Hypertension, April 1, 2008; 51(4): 862 - 866. [Abstract] [Full Text] [PDF] A. O. Spiel, J. C. Gilbert, and B. Jilma Von Willebrand Factor in Cardiovascular Disease: Focus on Acute Coronary Syndromes Circulation, March 18, 2008; 117(11): 1449 - 1459. [Abstract] [Full Text] [PDF] B. Lammle, J. A. Kremer Hovinga, and J. N. George Acquired thrombotic thrombocytopenic purpura: ADAMTS13 activity, anti-ADAMTS13 autoantibodies and risk of recurrent disease Haematologica, February 1, 2008; 93(2): 172 - 177. [Full Text] [PDF] R.-H. Huang, Y. Wang, R. Roth, X. Yu, A. R. Purvis, J. E. Heuser, E. H. Egelman, and J. E. Sadler Assembly of Weibel Palade body-like tubules from N-terminal domains of von Willebrand factor PNAS, January 15, 2008; 105(2): 482 - 487. [Abstract] [Full Text] [PDF] K. Shim, P. J. Anderson, E. A. Tuley, E. Wiswall, and J. Evan Sadler Platelet-VWF complexes are preferred substrates of ADAMTS13 under fluid shear stress Blood, January 15, 2008; 111(2): 651 - 657. [Abstract] [Full Text] [PDF] D. J. Metcalf, T. D. Nightingale, H. L. Zenner, W. W. Lui-Roberts, and D. F. Cutler Formation and function of Weibel-Palade bodies J. Cell Sci., January 1, 2008; 121(1): 19 - 27. [Abstract] [Full Text] [PDF] H. Choi, K. Aboulfatova, H. J. Pownall, R. Cook, and J.-f. Dong Shear-induced Disulfide Bond Formation Regulates Adhesion Activity of von Willebrand Factor J. Biol. Chem., December 7, 2007; 282(49): 35604 - 35611. [Abstract] [Full Text] [PDF] P. Zhang, W. Pan, A. H. Rux, B. S. Sachais, and X. L. Zheng The cooperative activity between the carboxyl-terminal TSP1 repeats and the CUB domains of ADAMTS13 is crucial for recognition of von Willebrand factor under flow Blood, September 15, 2007; 110(6): 1887 - 1894. [Abstract] [Full Text] [PDF] K. C. Desch and D. G. Motto Thrombotic Thrombocytopenic Purpura in Humans and Mice Arterioscler. Thromb. Vasc. Biol., September 1, 2007; 27(9): 1901 - 1908. [Abstract] [Full Text] [PDF] Z. M. Ruggeri and G. L. Mendolicchio Adhesion Mechanisms in Platelet Function Circ. Res., June 22, 2007; 100(12): 1673 - 1685. [Abstract] [Full Text] [PDF] H. L. Zenner, L. M. Collinson, G. Michaux, and D. F. Cutler High-pressure freezing provides insights into Weibel-Palade body biogenesis J. Cell Sci., June 15, 2007; 120(12): 2117 - 2125. [Abstract] [Full Text] [PDF] T. C. Nguyen, A. Liu, L. Liu, C. Ball, H. Choi, W. S. May, K. Aboulfatova, A. L. Bergeron, and J.-f. Dong Acquired ADAMTS-13 deficiency in pediatric patients with severe sepsis Haematologica, January 1, 2007; 92(1): 121 - 124. [Abstract] [Full Text] [PDF] S. M. T. Serrano, J. Kim, D. Wang, B. Dragulev, J. D. Shannon, H. H. Mann, G. Veit, R. Wagener, M. Koch, and J. W. Fox The Cysteine-rich Domain of Snake Venom Metalloproteinases Is a Ligand for von Willebrand Factor A Domains: ROLE IN SUBSTRATE TARGETING* J. Biol. Chem., December 29, 2006; 281(52): 39746 - 39756. [Abstract] [Full Text] [PDF] J.-J. Wu, K. Fujikawa, B. A. McMullen, and D. W. Chung Characterization of a core binding site for ADAMTS-13 in the A2 domain of von Willebrand factor PNAS, December 5, 2006; 103(49): 18470 - 18474. [Abstract] [Full Text] [PDF] J. A. Lopez Sticky business: von Willebrand factor in inflammation Blood, December 1, 2006; 108(12): 3627 - 3627. [Full Text] [PDF] R. De Cristofaro, F. Peyvandi, L. Baronciani, R. Palla, S. Lavoretano, R. Lombardi, E. Di Stasio, A. B. Federici, and P. M. Mannucci Molecular Mapping of the Chloride-binding Site in von Willebrand Factor (VWF): ENERGETICS AND CONFORMATIONAL EFFECTS ON THE VWF/ADAMTS-13 INTERACTION J. Biol. Chem., October 13, 2006; 281(41): 30400 - 30411. [Abstract] [Full Text] [PDF] D. Shang, X. W. Zheng, M. Niiya, and X. L. Zheng 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, October 1, 2006; 108(7): 2207 - 2215. [Abstract] [Full Text] [PDF] Z. M. Ruggeri, J. N. Orje, R. Habermann, A. B. Federici, and A. J. Reininger Activation-independent platelet adhesion and aggregation under elevated shear stress Blood, September 15, 2006; 108(6): 1903 - 1910. [Abstract] [Full Text] [PDF] C.-j. Liu, W. Kong, K. Xu, Y. Luan, K. Ilalov, B. Sehgal, S. Yu, R. D. Howell, and P. E. Di Cesare ADAMTS-12 Associates with and Degrades Cartilage Oligomeric Matrix Protein J. Biol. Chem., June 9, 2006; 281(23): 15800 - 15808. [Abstract] [Full Text] [PDF] G. Michaux, T. J. Pullen, S. L. Haberichter, and D. F. Cutler P-selectin binds to the D'-D3 domains of von Willebrand factor in Weibel-Palade bodies Blood, May 15, 2006; 107(10): 3922 - 3924. [Abstract] [Full Text] [PDF] Y. Shibagaki, M. Matsumoto, K. Kokame, S. Ohba, T. Miyata, Y. Fujimura, and T. Fujita Novel compound heterozygote mutations (H234Q/R1206X) of the ADAMTS13 gene in an adult patient with Upshaw-Schulman syndrome showing predominant episodes of repeated acute renal failure Nephrol. Dial. Transplant., May 1, 2006; 21(5): 1289 - 1292. [Abstract] [Full Text] [PDF] A. K. Chauhan, D. G. Motto, C. B. Lamb, W. Bergmeier, M. Dockal, B. Plaimauer, F. Scheiflinger, D. Ginsburg, and D. D. Wagner Systemic antithrombotic effects of ADAMTS13 J. Exp. Med., March 20, 2006; 203(3): 767 - 776. [Abstract] [Full Text] [PDF] M. A. Cruz Could shear stress be the answer? Blood, March 15, 2006; 107(6): 2218 - 2218. [Full Text] [PDF] R. Donadelli, J. N. Orje, C. Capoferri, G. Remuzzi, and Z. M. Ruggeri Size regulation of von Willebrand factor-mediated platelet thrombi by ADAMTS13 in flowing blood Blood, March 1, 2006; 107(5): 1943 - 1950. [Abstract] [Full Text] [PDF] A. Bonnefoy, K. Daenens, H. B. Feys, R. De Vos, P. Vandervoort, J. Vermylen, J. Lawler, and M. F. Hoylaerts Thrombospondin-1 controls vascular platelet recruitment and thrombus adherence in mice by protecting (sub)endothelial VWF from cleavage by ADAMTS13 Blood, February 1, 2006; 107(3): 955 - 964. [Abstract] [Full Text] [PDF] S. Zanardelli, J. T. B. Crawley, C. K. N. C. K. Chion, J. K. Lam, R. J. S. Preston, and D. A. Lane ADAMTS13 Substrate Recognition of von Willebrand Factor A2 Domain J. Biol. Chem., January 20, 2006; 281(3): 1555 - 1563. [Abstract] [Full Text] [PDF] T. Ono, J. Mimuro, S. Madoiwa, K. Soejima, Y. Kashiwakura, A. Ishiwata, K. Takano, T. Ohmori, and Y. Sakata 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, January 15, 2006; 107(2): 528 - 534. [Abstract] [Full Text] [PDF] S. P. Tull, S. I. Anderson, S. C. Hughan, S. P. Watson, G. B. Nash, and G. E. Rainger Cellular Pathology of Atherosclerosis: Smooth Muscle Cells Promote Adhesion of Platelets to Cocultured Endothelial Cells Circ. Res., January 6, 2006; 98(1): 98 - 104. [Abstract] [Full Text] [PDF] K. Soejima, H. Nakamura, M. Hirashima, W. Morikawa, C. Nozaki, and T. Nakagaki Analysis on the Molecular Species and Concentration of Circulating ADAMTS13 in Blood J. Biochem., January 1, 2006; 139(1): 147 - 154. [Abstract] [Full Text] [PDF] Z. Tao, Y. Peng, L. Nolasco, S. Cal, C. Lopez-Otin, R. Li, J. L. Moake, J. A. Lopez, and J.-f. Dong Recombinant CUB-1 domain polypeptide inhibits the cleavage of ULVWF strings by ADAMTS13 under flow conditions Blood, December 15, 2005; 106(13): 4139 - 4145. [Abstract] [Full Text] [PDF] L. H. Nolasco, N. A. Turner, A. Bernardo, Z. Tao, T. G. Cleary, J.-f. Dong, and J. L. Moake Hemolytic uremic syndrome-associated Shiga toxins promote endothelial-cell secretion and impair ADAMTS13 cleavage of unusually large von Willebrand factor multimers Blood, December 15, 2005; 106(13): 4199 - 4209. [Abstract] [Full Text] [PDF] J. J. J. Hulstein, P. G. de Groot, K. Silence, A. Veyradier, R. Fijnheer, and P. J. Lenting A novel nanobody that detects the gain-of-function phenotype of von Willebrand factor in ADAMTS13 deficiency and von Willebrand disease type 2B Blood, November 1, 2005; 106(9): 3035 - 3042. [Abstract] [Full Text] [PDF] J. Ai, P. Smith, S. Wang, P. Zhang, and X. L. Zheng The Proximal Carboxyl-terminal Domains of ADAMTS13 Determine Substrate Specificity and Are All Required for Cleavage of von Willebrand Factor J. Biol. Chem., August 19, 2005; 280(33): 29428 - 29434. [Abstract] [Full Text] [PDF] M. Rieger, P. M. Mannucci, J. A. K. Hovinga, A. Herzog, G. Gerstenbauer, C. Konetschny, K. Zimmermann, I. Scharrer, F. Peyvandi, M. Galbusera, et al. ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases Blood, August 15, 2005; 106(4): 1262 - 1267. [Abstract] [Full Text] [PDF] G. G. Levy, D. G. Motto, and D. Ginsburg ADAMTS13 turns 3 Blood, July 1, 2005; 106(1): 11 - 17. [Abstract] [Full Text] [PDF] Z. Tao, Y. Wang, H. Choi, A. Bernardo, K. Nishio, J. E. Sadler, J. A. Lopez, and J.-f. Dong Cleavage of ultralarge multimers of von Willebrand factor by C-terminal-truncated mutants of ADAMTS-13 under flow Blood, July 1, 2005; 106(1): 141 - 143. [Abstract] [Full Text] [PDF] E. M. Majerus, P. J. Anderson, and J. E. Sadler Binding of ADAMTS13 to von Willebrand Factor J. Biol. Chem., June 10, 2005; 280(23): 21773 - 21778. [Abstract] [Full Text] [PDF] M. Noris, S. Bucchioni, M. Galbusera, R. Donadelli, E. Bresin, F. Castelletti, J. Caprioli, S. Brioschi, F. Scheiflinger, G. Remuzzi, et al. Complement Factor H Mutation in Familial Thrombotic Thrombocytopenic Purpura with ADAMTS13 Deficiency and Renal Involvement J. Am. Soc. Nephrol., May 1, 2005; 16(5): 1177 - 1183. [Abstract] [Full Text] [PDF] J.-D. Studt, J. A. K. Hovinga, G. Antoine, M. Hermann, M. Rieger, F. Scheiflinger, and B. Lammle Fatal congenital thrombot