• 1
    Furlan M. Von Willebrand factor: molecular size and functional activity. Ann Hematol 1996; 72: 3418.DOI: 10.1007/s002770050184
  • 2
    Ruggeri ZM. Structure and function of von Willebrand factor. Thromb Haemost 1999; 82: 57684.
  • 3
    Sadler JE. Biochemistry and genetics of von Willebrand factor. Annu Rev Biochem 1998; 67: 395424.DOI: 10.1146/annurev.biochem.67.1.395
  • 4
    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: 145661.
  • 5
    Federici AB, Bader R, Pagani S, Colibretti ML, De Marco L, Mannucci PM. Binding of von Willebrand factor to glycoproteins Ib and IIb/IIIa complex: affinity is related to multimeric size. Br J Haematol 1989; 73: 939.
  • 6
    Arya M, Anvari B, Romo GM, Cruz MA, Dong JF, McIntire LV, Moake JL, Lopez JA. Ultra-large multimers of von Willebrand factor form spontaneous high-strength bonds with the platelet GP Ib-IX complex: studies using optical tweezers. Blood 2002; 99: 39717.
  • 7
    Moake JL, Rudy CK, Troll JH, Weinstein MJ, Colannino NM, Azocar J, Seder RH, Hong SL, Deykin D. Unusually large plasma factor VIII:von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med 1982; 307: 14325.
  • 8
    Moschcowitz E. Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc NY Pathol Soc 1924; 24: 214.
  • 9
    Lankford KV, Hillyer CD. Thrombotic thrombocytopenic purpura: new insights in disease pathogenesis and therapy. Transfus Med Rev 2000; 14: 24457.
  • 10
    Bukowski RM. Thrombotic thrombocytopenic purpura: a review. Prog Hemost Thromb 1982; 6: 287337.
  • 11
    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: 422334.
  • 12
    Tsai HM. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood 1996; 87: 423544.
  • 13
    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: 283946.
  • 14
    Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998; 339: 158594.DOI: 10.1056/NEJM199811263392203
  • 15
    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: 3097103.
  • 16
    Furlan M, Lämmle B. Deficiency of von Willebrand factor-cleaving protease in familial and acquired thrombotic thrombocytopenic purpura. Baillieres Clin Haematol 1998; 11: 50914.
  • 17
    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: 16626.
  • 18
    Gerritsen HE, Robles R, Lämmle B, Furlan M. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood 2001; 98: 165461.
  • 19
    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: 4105963.DOI: 10.1074/jbc.C100515200
  • 20
    Soejima K, Mimura N, Hirashima M, Maeda H, Hamamoto T, Nakagaki T, Nozaki C. 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: 47580.
  • 21
    Levy GG, Nichols WC, Lian EC, Foroud T, McClintick JN, McGee BM, Yang AY, Siemieniak DR, Stark KR, Gruppo R, Sarode R, Shurin SB, Chandrasekaran V, Stabler SP, Sabio H, Bouhassira EE, Upshaw JD Jr, Ginsburg D, Tsai HM. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 2001; 413: 48894.DOI: 10.1038/35097008
  • 22
    Tang BL, Hong W. ADAMTS: a novel family of proteases with an ADAM protease domain and thrombospondin 1 repeats. FEBS Lett 1999; 445: 2235.DOI: 10.1016/S0014-5793(99)00119-2
  • 23
    Tang BL. ADAMTS: a novel family of extracellular matrix proteases. Int J Biochem Cell Biol 2001; 33: 3344.DOI: 10.1016/S1357-2725(00)00061-3
  • 24
    Kokame K, Matsumoto M, Soejima K, Yagi H, Ishizashi H, Funato M, Tamai H, Konno M, Kamide K, Kawano Y, Miyata T, Fujimura Y. Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity. Proc Natl Acad Sci USA 2002; 99: 119027.DOI: 10.1073/pnas.172277399
  • 25
    Plaimauer B, Zimmermann K, Völkel D, Antoine G, Kerschbaumer R, Jenab P, Furlan M, Gerritsen H, Lämmle B, Schwarz HP, Scheiflinger F. Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13). Blood 2002; 100: 362632.
  • 26
    Antoine G, Zimmermann K, Plaimauer B, Grillowitzer M, Studt JD, Lämmle B, Scheiflinger F. ADAMTS13 gene defects in two brothers with constitutional thrombotic thrombocytopenic purpura and normalization of von Willebrand factor-cleaving protease activity by recombinant human ADAMTS13. Br J Haematol 2003; 120: 8214.DOI: 10.1046/j.1365-2141.2003.04183.x
  • 27
    Moake JL. Thrombotic microangiopathies. N Engl J Med 2002; 347: 589600.DOI: 10.1056/NEJMra020528
  • 28
    Banno F, Kaminaka K, Soejima K, Kokame K, Miyata T. Identification of strain-specific variants of mouse Adamts13 gene encoding von Willebrand factor-cleaving protease. J Biol Chem 2004; 279: 30896903.
  • 29
    Kozak M. An analysis of 5′-noncoding sequences from 699 vertebrate messenger RNAs. Nucl Acids Res 1987; 15: 812548.
  • 30
    Schlokat U, Fischer BE, Herlitschka S, Antoine G, Preininger A, Mohr G, Himmelspach M, Kistner O, Falkner FG, Dorner F. Production of highly homogeneous and structurally intact recombinant von Willebrand factor multimers by furin-mediated propeptide removal in vitro. Biotechnol Appl Biochem 1996; 24: 25767.
  • 31
    Bohm M, Vigh T, Scharrer I. Evaluation and clinical application of a new method for measuring activity of von Willebrand factor-cleaving metalloprotease (ADAMTS13). Ann Hematol 2002; 81: 4305.DOI: 10.1007/s00277-002-0502-3
  • 32
    Ruggeri ZM, Zimmerman TS. The complex multimeric composition of factor VIII/von Willebrand factor. Blood 1981; 57: 11403.
  • 33
    Majerus EM, Zheng X, Tuley EA, Sadler JE. Cleavage of the ADAMTS13 propeptide is not required for protease activity. J Biol Chem 2003; 278: 466438.DOI: 10.1074/jbc.M309872200
  • 34
    Apte SS. A disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motifs: the ADAMTS family. Int J Biochem Cell Biol 2004; 36: 9815.DOI: 10.1016/j.biocel.2004.01.014
  • 35
    Ruoslahti E. RGD and other recognition sequences for integrins. Annu Rev Cell Dev Biol 1996; 12: 697715.DOI: 10.1146/annurev.cellbio.12.1.697
  • 36
    Hofsteenge J, Huwiler KG, Macek B, Hess D, Lawler J, Mosher DF, Peter-Katalinic J. C-mannosylation and O-fucosylation of the thrombospondin type 1 module. J Biol Chem 2001; 276: 648598.DOI: 10.1074/jbc.M008073200
  • 37
    Kokame K, Miyata T. Genetic defects leading to hereditary thrombotic thrombocytopenic purpura. Semin Hematol 2004; 41: 3440.DOI: 10.1053/j.seminhematol.2003.10.002
  • 38
    Kuno K, Iizasa H, Ohno S, Matsushima K. The exon/intron organization and chromosomal mapping of the mouse ADAMTS-1 gene encoding an ADAM family protein with TSP motifs. Genomics 1997; 46: 46671.DOI: 10.1006/geno.1997.5064
  • 39
    Colige A, Vandenberghe I, Thiry M, Lambert CA, Van Beeumen J, Li SW, Prockop DJ, Lapiere CM, Nusgens BV. Cloning and characterization of ADAMTS-14, a novel ADAMTS displaying high homology with ADAMTS-2 and ADAMTS-3. J Biol Chem 2002; 277: 575666.
  • 40
    Llamazares M, Cal S, Quesada V, Lopez-Otin C. Identification and characterization of ADAMTS-20 defines a novel subfamily of metalloproteinases-disintegrins with multiple thrombospondin-1 repeats and a unique GON domain. J Biol Chem 2003; 278: 133829.DOI: 10.1074/jbc.M211900200
  • 41
    Menke DB, Page DC. Sexually dimorphic gene expression in the developing mouse gonad. Gene Expr Patterns 2002; 2: 35967.DOI: 10.1016/S1567-133X(02)00022-4
  • 42
    Plaimauer B, Scheiflinger F. Expression and characterization of recombinant ADAMTS-13. Semin Hematol 2004; 41: 2433.DOI: 10.1053/j.seminhematol.2003.10.006