ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions

Blood

Volumn 100, Issue 12, 2002, Pages 4033-4039

  Dong, Jing fei ^a,b,c   Moake, Joel L ^a,b,c   Nolasco, Leticia ^a,b,c   Bernardo, Aubrey ^a,b,c   Arceneaux, Wendy ^a,b,c   Shrimpton, Corie N ^a,b,c   Schade, Alicia J ^a,b,c   McIntire, Larry V ^a,b,c   Fujikawa, Kazuo ^a,b,c   López, José A ^a,b,c  

^a BAYLOR COLLEGE OF MEDICINE   (United States)

^b UNIVERSITY OF WASHINGTON SCHOOL OF MEDICINE   (United States)

^c RICE UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

GLYCOPROTEIN; GLYCOPROTEIN IB; GLYCOPROTEIN IB IX V COMPLEX; METALLOPROTEINASE; POLYMER; PROTEIN; UNCLASSIFIED DRUG; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

ADULT; ANIMAL CELL; ARTICLE; BINDING SITE; CELL CULTURE; CELL SURFACE; CHO CELL; CLINICAL ARTICLE; CONTROLLED STUDY; ENDOTHELIUM CELL; ENZYME ACTIVITY; FEMALE; HEMOSTASIS; HUMAN; HUMAN CELL; MALE; NONHUMAN; PRIORITY JOURNAL; PROTEIN DEGRADATION; SHEAR STRESS; TENSILE STRENGTH; THROMBOCYTE; THROMBOCYTE ADHESION; THROMBOCYTE AGGREGATION; THROMBOGENESIS; THROMBOTIC THROMBOCYTOPENIC PURPURA; THROMBUS;

EID: 0036893186     PISSN: 00064971     EISSN: None     Source Type: Journal    
DOI: 10.1182/blood-2002-05-1401     Document Type: Article

References (29)

1. Sadler JE. Biochemistry and genetics of von Willebrand factor. Annu Rev Biochem. 1998;67:395-424.
2. Wagner DD, Marder VJ. Biosynthesis of von Willebrand protein by human endothelial cells. Identification of a large precursor polypeptide chain. J Biol Chem. 1983;258:2065-2067.
3. van de Ven WJ, Voorberg J, Fontijn R, et al. Furin is a subtilisin-like proprotein processing enzyme in higher eukaryotes. Mol Biol Rep. 1990;14:265-275.
4. Moake JL, Rudy CK, Troll JH, et al. Unusually large plasma factor VIII:von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med. 1982;307:1432-1435.
5. Zheng X, Chung D, Takayama TK, Majerus EM, Sadler JE, Fujikawa K. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem. 2001;276:41059-41063.
6. Fujikawa K, Suzuki H, McMullen B, Chung D. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood. 2001;98:1662-1666.
7. Gerritsen HE, Robles R, Lammle B, Furlan M. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood. 2001;98:1654-1661.
8. Furlan M, Robles R, Lamie B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996;87:4223-4234.
9. Dent JA, Galbusera M, Ruggeri ZM. Heterogeneity of plasma von Willebrand factor multimers resulting from proteolysis of the constituent subunit. J Clin Invest. 1991;88:774-782.
10. Furlan M. Von Willebrand factor: Molecular size and functional activity. Ann Hematol. 1996;72:341-348.
11. Allford SL, Machin SJ. Current understanding of the pathophysiology of thrombotic thrombocytopenic purpura. J Clin Pathol. 2000;53:497-501.
12. Baker KR, Moake JL. Thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. Curr Opin Pediatr. 2000;12:23-28.
13. Moake JL, McPherson PD. Abnormalities of von Willebrand factor multimers in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. Am J Med. 1989;87:9N-15N.
14. Moake JL, Turner NA, Stathopoulos NA, Nolasco LH, Hellums JD. Involvement of large plasma von Willebrand factor (vWF) multimers and unusually large vWF forms derived from endothelial cells in shear stress-induced platelet aggregation. J Clin Invest. 1986;78:1456-1461.
15. Takahashi K, Sawasaki Y, Hata J, Mukai K, Goto T. Spontaneous transformation and immortalization of human endothelial cells. In Vitro Cell Dev Biol. 1990;26:265-274.
16. Dong JF, Berndt MC, Schade A, McIntire LV, Andrews RK, López JA. Ristocetin-dependent, but not botrocetin-dependent, binding of von Willebrand factor to the platelet glycoprotein Ib-IX-V complex correlates with shear-dependent interactions. Blood. 2001;97:162-168.
17. Dong J, Ye P, Schade AJ, et al. Tyrosine sulfation of glycoprotein Ibα. Role of electrostatic interactions in von Willebrand factor binding. J Biol Chem. 2001;276:16690-16694.
18. Kroll MH, Hellums JD, McIntire LV, Schafer AI, Moake JL. Platelets and shear stress. Blood. 1996;88:1525-1541.
19. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998; 339:1585-1594.
20. Furlan M, Robles R, Galbusera M, et al. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolyticuremic syndrome. N Engl J Med. 1998;339:1578-1584.
21. Arya M, Anvari B, Romo GM, et al. Ultralarge multimers of von Willebrand factor form spontaneous high-strength bonds with the platelet glycoprotein Ib-IX complex: Studies using optical tweezers. Blood. 2002;99:3971-3977.
22. Savage B, Saldivar E, Ruggeri ZM. Initiation of platelet adhesion by arrest onto fibrinogen or translocation on von Willebrand factor. Cell. 1996; 84:289-297.
23. Fredrickson BJ, Dong JF, McIntire LV, López JA. Shear-dependent rolling on von Willebrand factor of mammalian cells expressing the platelet glycoprotein Ib-IX-V complex. Blood. 1998;92:3684-3693.
24. Dong J, Schade AJ, Romo GM, et al. Novel gain-of-function mutations of platelet glycoprotein Ibα by valine mutagenesis in the Cys209-Cys248 disulfide loop. Functional analysis under statis and dynamic conditions. J Biol Chem. 2000;275:27663-27670.
25. Moake JL. Thrombotic thrombocytopenic purpura: The systemic clumping "plague." Annu Rev Med. 2002;53:75-88.
26. Savage B, Sixma JJ, Ruggeri ZM. Functional self-association of von Willebrand factor during platelet adhesion under flow. Proc Natl Acad Sci U S A. 2002;99:425-430.
27. Andre P, Denis CV, Ware J, et al. Platelets adhere to and translocate on von Willebrand factor presented by endothelium in stimulated veins. Blood. 2000;96:3322-3328.
28. Tandon NN, Rock G, Jamieson GA. Anti-CD36 antibodies in thrombotic thrombocytopenic purpura. Br J Haematol. 1994;88:816-825.
29. Savasan S, Taub JW, Buck S, Botterill M, Furlan M, Ravindranath Y. Congenital microangiopathic hemolytic anemia and thrombocytopenia with unusually large von Willebrand factor multimers and von Willebrand factor-cleaving protease. J Pediatr Hematol Oncol. 2001;23:364-367.