Von Willebrand factor cleaved from endothelial cells by ADAMTS13 remains ultralarge in size

Journal of Thrombosis and Haemostasis

Volumn 7, Issue 10, 2009, Pages 1749-1752

  Jin, S Y ^a   Skipwith, C G ^a   Shang, D ^a   Zheng, X L ^a,b  

^a CHILDREN S HOSPITAL OF PHILADELPHIA   (United States)

^b HOSPITAL OF THE UNIVERSITY OF PENNSYLVANIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

CONTROLLED STUDY; ENDOTHELIUM CELL; GENE MUTATION; HEMOSTASIS; HUMAN; HUMAN CELL; INFLAMMATION; LETTER; PARTICLE SIZE; PRIORITY JOURNAL; PROTEIN CLEAVAGE; PROTEIN SECRETION; PROTEIN SYNTHESIS; SHEAR STRESS;

ADAM PROTEINS; CULTURE MEDIA, CONDITIONED; ENDOTHELIAL CELLS; HEMORHEOLOGY; HISTAMINE; HUMANS; MOLECULAR WEIGHT; PEPTIDE FRAGMENTS; PROTEIN BINDING; PROTEIN PROCESSING, POST-TRANSLATIONAL; PROTEIN STRUCTURE, TERTIARY; RECOMBINANT PROTEINS; STRESS, MECHANICAL; STRUCTURE-ACTIVITY RELATIONSHIP; VON WILLEBRAND FACTOR; WEIBEL-PALADE BODIES;

EID: 70350015029     PISSN: 15387933     EISSN: 15387836     Source Type: Journal    
DOI: 10.1111/j.1538-7836.2009.03570.x     Document Type: Letter

References (20)

1. Wagner DD, Marder VJ. Biosynthesis of von Willebrand protein by human endothelial cells: processing steps and their intracellular localization. J Cell Biol 1984, 99:2123-30.
2. Padilla A, Moake JL, Bernardo A, Ball C, Wang Y, Arya M, Nolasco L, Turner N, Berndt MC, Anvari B, Lopez JA, Dong JF. P-selectin anchors newly released ultra-large von Willebrand factor multimers to the endothelial cell surface. Blood 2004, 103:2150-6.
3. Huang J, Roth R, Heuser JE, Sadler JE. Integrin {alpha}v{beta}3 on human endothelial cells binds von Willebrand factor strings under fluid shear stress. Blood 2008, 113:1589-697.
4. Sadler JE. von Willebrand factor: two sides of a coin. J Thromb Haemost 2005, 3:1702-9.
5. Dong JF, Moake JL, Nolasco L, Bernardo A, Arceneaux W, Shrimpton CN, Schade AJ, McIntire LV, Fujikawa K, Lopez JA. ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions. Blood 2002, 100:4033-9.
6. Dong JF, Moake JL, Bernardo A, Fujikawa K, Ball C, Nolasco L, Lopez JA, Cruz MA. ADAMTS-13 metalloprotease interacts with the endothelial cell-derived ultra-large von Willebrand factor. J Biol Chem 2003, 278:29633-9.
7. Moake JL. Thrombotic thrombocytopenic purpura: the systemic clumping 'plague'. Annu Rev Med 2002, 53:75-88.
8. Chauhan AK, Kisucka J, Brill A, Walsh MT, Scheiflinger F, Wagner DD. ADAMTS13: a new link between thrombosis and inflammation. J Exp Med 2008, 205:2065-74.
9. Furlan M, Robles R, Lammle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood 1996, 87:4223-34.
10. Tsai HM. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood 1996, 87:4235-44.
11. Tsai HM, Sussman I, Nagel RL. Shear stress enhances the proteolysis of von Willebrand factor in normal plasma. Blood 1994, 83:2171-9.
12. Zhang P, Pan W, Rux AH, Sachais BS, Zheng XL. The cooperative activity between the carboxyl-terminal TSP-1 repeats and the CUB domains of ADAMTS13 is crucial for recognition of von Willebrand factor under flow. Blood 2007, 110:1887-94.
13. Chauhan AK, Motto DG, Lamb CB, Bergmeier W, Dockal M, Plaimauer B, Scheiflinger F, Ginsburg D, Wagner DD. Systemic antithrombotic effects of ADAMTS13. J Exp Med 2006, 203:767-76.
14. Turner N, Nolasco L, Dong JF, Moake J. ADAMTS-13 cleaves long von Willebrand factor multimeric strings anchored to endothelial cells in the absence of flow, platelets or conformation-altering chemicals. J Thromb Haemost 2009, 7:229-32.
15. Niiya M, Endo M, Shang D, Zoltick PW, Muvarak NE, Cao W, Jin SY, Skipwith CG, Motto DG, Flake AW, Zheng XL. Correction of ADAMTS13 deficiency by in utero gene transfer of lentiviral vector encoding ADAMTS13 genes. Mol Ther 2009, 17:34-41.
16. Gao W, Anderson PJ, Majerus EM, Tuley EA, Sadler JE. Exosite interactions contribute to tension-induced cleavage of von Willebrand factor by the antithrombotic ADAMTS13 metalloprotease. Proc Natl Acad Sci USA 2006, 103:19099-104.
17. Zheng XL, Nishio K, Majerus EM, Sadler JE. Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13. J Biol Chem 2003, 278:30136-41.
18. Raife TJ, Cao W, Atkinson BS, Bedell B, Montgomery RR, Lentz SR, Johnson GF, Zheng XL. Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site. Blood 2009, 114:1666-74.
19. Cao WJ, Krishnaswamy S, Camire RM, Lenting PJ, Zheng XL. Factor VIII accelerates proteolytic cleavage of von Willebrand factor by ADAMTS13. Proc Natl Acad Sci USA 2008, 105:7416-21.
20. Shim K, Anderson PJ, Tuley EA, Wiswall E, Sadler JE. Platelet-VWF complexes are preferred substrates of ADAMTS13 under fluid shear stress. Blood 2007, 111:651-7.