ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage

Blood

Volumn 102, Issue 9, 2003, Pages 3232-3237

  Soejima, Kenji ^a   Matsumoto, Masanori ^b   Kokame, Koichi ^b   Yagi, Hideo ^b   Ishizashi, Hiromichi ^b   Maeda, Hiroaki ^b   Nozaki, Chikateru ^b   Miyata, Toshiyuki ^b   Fujimura, Yoshihiro ^b   Nakagaki, Tomohiro ^b  

^a Chemo Sero Therapeutic Research Institute   (Japan)

^b NONE

Author keywords

[No Author keywords available]

Indexed keywords

IMMUNOGLOBULIN G; METALLOPROTEINASE; THROMBOSPONDIN; UNCLASSIFIED DRUG; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

ARTICLE; CARBOXY TERMINAL SEQUENCE; CONTROLLED STUDY; EPITOPE MAPPING; HUMAN; HUMAN CELL; POINT MUTATION; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN DOMAIN; THROMBOTIC THROMBOCYTOPENIC PURPURA;

EID: 0142152440     PISSN: 00064971     EISSN: None     Source Type: Journal    
DOI: 10.1182/blood-2003-03-0908     Document Type: Article

References (40)

1. Zimmerman TS, Ruggeri ZM. von Willebrand disease. Hum Pathol. 1987;18:140-152.
2. Jaffe EA, Hoyer LW, Nachman RL. Synthesis of von Willebrand factor by cultured human endothelial cells. Proc Natl Acad Sci U S A. 1974;71: 1906-1909.
3. Fujimura Y, Titani K. Structure and function of von Willebrand factor. In: Bloom AL, Forbes CD, Thomas DP, Tuddenham EGD, eds. Haemostasis and Thrombosis. New York, NY: Churchill Livingstone; 1994:379-395.
4. Ruggeri ZM. von Willebrand factor. J Clin invest. 1997;99:559-564.
5. Sadler JE. Biochemistry and genetics of von Willebrand factor. Annu Rev Biochem. 1998;67:395-424.
6. Ruggeri ZM, Zimmerman TS. The complex multimeric composition of factor VIII/von Willebrand factor. Blood. 1981;57:1140-1143.
7. Sixma JJ, Sakariassen KS, Beeser-Visser NH, Ottenhof-Rovers M, Bolhuis PA. Adhesion of platelets to human artery subendothelium: effect of factor VIII-von Wiiiebrand factor of various multimeric composition. Blood. 1984;63:128-139.
8. Sporn LA, Marder VJ, Wagner DD. von Willebrand factor released from Weibel-Palade bodies binds more avidly to extracellular matrix than that secreted constitutively. Blood. 1987;69:1531-1534.
9. Zimmerman TS, Dent JA, Ruggeri ZM, Nannini LH. Subunit composition of plasma von Willebrand factor: cleavage is present in normal individuals, increased in IIA and IIB von Willebrand disease, but minimal in variants with aberrant structure of individual oligomers (types IIC, IID, and IIE). J Clin Invest. 1986;77:947-951.
10. Berkowitz SD, Dent J, Roberts J, et al. Epitope mapping of the von Willebrand factor subunit distinguishes fragments present in normal and type IIA von Willebrand disease from those generated by piasmin. J Clin Invest. 1987;79:524-531.
11. 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.
12. Furlan M, Robles R, Affolter D, Meyer D, Baillod P, Lämmle B. Triplet structure of von Willebrand factor reflects proteolytic degradation of high molecular weight multimers. Proc Natl Acad Sci U S A. 1993;90:7503-7507.
13. Dent JA, Berkowitz SD, Ware J, Kasper CK, Ruggeri ZM. Identification of a cleavage site directing the immunochemical detection of molecular abnormalities in type IIA von Willebrand factor. Proc Natl Acad Sci U S A. 1990;87:6306-6310.
14. 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.
15. Moschcowitz E. Hyalin thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc N Y Pathol Soc. 1924;24:21-24.
16. Furlan M, Robles R, Lämmle 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.
17. Tsai H-M. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood. 1996;87:4235-4244.
18. Furlan M, Robles R, Solenthaler M, Wassmer M, Sandoz P, Lämmle B. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood. 1997;89:3097-3103.
19. Kinoshita S, Yoshioka A, Park YD, et al. Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura. Int J Hematol. 2001;74:101-108.
20. Furlan M, Robles R, Galbusera M, et al. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med. 1998;339:1578-1584.
21. Furlan M, Robles R, Solenthaler M, Lämmle B. Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura. Blood. 1998;91:2839-2846.
22. Tsai H-M, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998; 339:1585-1594.
23. Gerritsen HE, Robles R, Lämmle B, Furlan M. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood. 2001;98: 1654-1661.
24. 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.
25. Soejima K, Mimura N, Hirashima M, et al. A novel human metalloprotease synthesized in the liver and secreted into the blood: possibly, the von Willebrand factor-cleaving protease? J Biochem (Tokyo). 2001;130:475-480.
26. 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.
27. Levy GG, Nichols WC, Lian EC, et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. 2001;413:488-494.
28. Cal S, Obaya AJ, Llamazares M, Garabaya C, Quesada V, Lopez-Otin C. Cloning, expression analysis, and structural characterization of seven novel human ADAMTSs, a family of metalloproteinases with disintegrin and thrombospondin-1 domains. Gene. 2002;283:49-62.
29. Plaimauer B, Zimmermann K, Volkel D, et al. Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13). Blood. 2002;100:3626-3632.
30. Matsumoto M, Chisuwa H, Nakazawa Y, et al. Living-related liver transplantation rescues reduced vWF-cleaving protease activity in patients with cirrhotic biliary atresia [abstract]. Blood. 2000;96:636a.
31. Dong JF, Moake JL, Nolasco L, et al. ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions. Blood. 2002;100:4033-4039.
32. Ruoslahti E, Pierschbacher MD. Arg-Gly-Asp: a versatile cell recognition signal. Cell. 1986;44: 517-518.
33. Kokame K, Matsumoto M, Soejima K, et al. Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity. Proc Nat Acad Sci U S A. 2002;99:11902-11907.
34. Niwa H, Yamamura K, Miyazaki J. Efficient selection for high-expression transfectants with a novel eukaryotic vector. Gene. 1991;108:193-199.
35. Soejima K, Mizuguchi J, Yuguchi M, Nakagaki T, Higashi S, Iwanaga S. Factor VIIa modified in the 170 loop shows enhanced catalytic activity but does not change the zymogen-like property. J Biol Chem. 2001;276:17229-17235.
36. Zheng X, Nishio K, Majerus E, Sadler JE. Characterization of recombinant ADAMTS-13 in COS7 cells and identification of domains required for cleavage of von Willebrand factor [abstract]. Blood. 2002;100:256a.
37. Schneppenheim R, Budde U, Oyen F, et al. Von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP. Blood. 2003;101:1845-1850.
38. Kuno K, Matsushima K. ADAMTS-1 protein anchors at the extracellular matrix through the thrombospondin type I motifs and its spacing region. J Biol Chem. 1998;273:13912-13917.
39. Flannery CR, Zeng W, Corcoran C, et al. Auto-catalytic cleavage of ADAMTS-4 (Aggrecanase-1) reveals multiple glycosaminoglycan-binding sites. J Biol Chem. 2002;277:42775-42780.
40. Smith KM, Gaultier A, Cousin H, Alfandari D, White JM, DeSimone DW. The cysteine-rich domain regulates ADAM protease function in vivo. J Cell Biol. 2002;159:893-902.