Purification of human von Willebrand factor–cleaving protease and its identification as a new member of the metalloproteinase family

Blood - Tập 98 Số 6 - Trang 1662-1666 - 2001
Kazuo Fujikawa1, Hiroshi Suzuki1, Brad A. McMullen1, Dominic W. Chung1
1From the Department of Biochemistry, University of Washington, Seattle.

Tóm tắt

Abstract von Willebrand factor (vWF) is synthesized in megakaryocytes and endothelial cells as a very large multimer, but circulates in plasma as a group of multimers ranging from 500 to 10 000 kd. An important mechanism for depolymerization of the large multimers is the limited proteolysis by a vWF-cleaving protease present in plasma. The absence or inactivation of the vWF-cleaving protease results in the accumulation of large multimers, which may cause thrombotic thrombocytopenic purpura. The vWF-cleaving protease was first described as a Ca++-dependent proteinase with an apparent molecular weight of approximately 300 kd. Thus far, however, it has not been isolated and characterized. In this study, the purification of human vWF-cleaving protease from a commercial preparation of factor VIII/vWF concentrate by means of several column chromatographic steps, including 2 steps of heparin-Sepharose column, is reported. Sodium dodecyl sulfate–polyacrylamide gel electrophoresis analysis of the anion exchange and gel filtration column fractions showed that the vWF-cleaving protease activity corresponded to a protein band of 150 kd. After reduction, it migrated with an apparent weight of 190 kd. The amino terminal sequence of the 150-kd band was AAGGIL(H)LE(L)L(D)AXG(P)X(V)XQ (single-letter amino acid codes), with the tentative residues shown in parentheses. A search of the human genome sequence identified the vWF-cleaving protease as a new member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin type I motif) family of metalloproteinase. An active site sequence of HEIGHSFGLEHE (single-letter amino acid codes) was located at 150 residues from the N terminus of the protein.

Từ khóa


Tài liệu tham khảo

Sadler, 1998, Biochemistry and genetics of von Willebrand factor., Annu Rev Biochem., 67, 395, 10.1146/annurev.biochem.67.1.395

Ruggeri, 1992, The structure and function of von Willebrand factor., Thromb Haemost., 67, 594, 10.1055/s-0038-1648506

Sadler, 1985, Cloning and characterization of two cDNAs coding for human von Willebrand factor., Proc Natl Acad Sci U S A., 82, 6394, 10.1073/pnas.82.19.6394

Titani, 1986, Amino acid sequence of human von Willebrand factor., Biochemistry., 25, 3171, 10.1021/bi00359a015

Jaffe, 1974, Synthesis of von Willebrand factor by cultured human endothelial cells., Proc Natl Acad Sci U S A., 71, 1906, 10.1073/pnas.71.5.1906

Nachman, 1977, Synthesis of factor VIII antigen by cultured guinea pig megakaryocytes., J Clin Invest., 60, 914, 10.1172/JCI108846

Wagner, 1987, Topology and order of formation of interchain disulfide bonds in von Willebrand factor., Blood., 69, 27, 10.1182/blood.V69.1.27.27

Girma, 1986, Limited proteolysis of human von Willebrand factor by Staphylococcus aureus V-8 protease: isolation and partial characterization of a platelet-binding domain., Biochemistry., 25, 3156, 10.1021/bi00359a013

Wagner, 1982, Immunolocalization of von Willebrand protein in Weibel-Palade bodies of human endothelial cells., J Cell Biol., 95, 355, 10.1083/jcb.95.1.355

Sporn, 1986, Inducible secretion of large, biologically potent von Willebrand factor multimers., Cell., 46, 185, 10.1016/0092-8674(86)90735-X

Tsai, 1989, Multimeric composition of endothelial cell-derived von Willebrand factor., Blood., 73, 2074, 10.1182/blood.V73.8.2074.2074

Ruggeri, 1982, Multimeric composition of factor VIII/von Willebrand factor following administration of DDAVP: implications for pathophysiology and therapy of von Willebrand's disease subtypes., Blood., 59, 1272, 10.1182/blood.V59.6.1272.1272

Furlan, 1996, Von Willebrand factor: molecular size and functional activity., Ann Hematol., 72, 341, 10.1007/s002770050184

Moake, 1997, Studies on the pathophysiology of thrombotic thrombocytopenic purpura., Semin Hematol., 43, 83

Dent, 1991, Heterogeneity of plasma von Willebrand factor multimers resulting from proteolysis of the constituent subunit., J Clin Invest., 88, 774, 10.1172/JCI115376

Tsai, 1991, Subunit composition of plasma von Willebrand factor multimers: evidence for a non-proteolytic mechanism resulting in apparent increase in proteolytic fragments., Thromb Res., 63, 179, 10.1016/0049-3848(91)90280-A

Furlan, 1993, Triplet structure of von Willebrand factor reflects proteolytic degradation of high molecular weight multimers., Proc Natl Acad Sci U S A., 90, 7503, 10.1073/pnas.90.16.7503

Furlan, 1996, Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis., Blood., 87, 4223, 10.1182/blood.V87.10.4223.bloodjournal87104223

Tsai, 1994, Shear stress enhances the proteolysis of von Willebrand factor in normal plasma., Blood., 83, 2171, 10.1182/blood.V83.8.2171.2171

Furlan, 1997, Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura., Blood., 89, 3097, 10.1182/blood.V89.9.3097

Furlan, 1998, von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome., N Engl J Med., 339, 1578, 10.1056/NEJM199811263392202

Tsai, 1998, Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura., N Engl J Med., 339, 585, 10.1056/NEJM199811263392203

van der Plas, 1999, von Willebrand factor proteolysis is deficient in classic, but not in bone marrow transplantation-associated, thrombotic thrombocytopenic purpura., Blood., 93, 3798, 10.1182/blood.V93.11.3798

Fujikawa, 1973, Isolation and charcterization of bovine factor IX (Christmas factor)., Biochemistry., 12, 4938, 10.1021/bi00748a019

Shapiro, 1973, The subunit structure of normal and hemophilic factor VIII., J Clin Invest., 52, 2198, 10.1172/JCI107405

Laemmli, 1970, Cleavage of structural proteins during the assembly of the head of bacteriophage T4., Nature., 227, 680, 10.1038/227680a0

Towbin, 1979, Electrophoretic transfer of proteins from polyacrylamide gels to nitrocellulose sheets: procedure and some applications., Proc Natl Acad Sci U S A., 76, 4350, 10.1073/pnas.76.9.4350

Zacharius, 1969, Glycoprotein staining following electorphoresis on acrylamide gels., Anal Biochem., 30, 148, 10.1016/0003-2697(69)90383-2

Caterson, 2000, Mechanisms involved in cartilage proteoglycan catabolism., Matrix Biol., 19, 333, 10.1016/S0945-053X(00)00078-0

Wolfsberg, 1995, ADAM, a widely distributed and developmentally regulated gene family encoding membrane proteins with a disintegrin and metalloprotease domain., Dev Biol., 169, 378, 10.1006/dbio.1995.1152

Tsai, 1996, Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion., Blood., 87, 4235, 10.1182/blood.V87.10.4235.bloodjournal87104235

Gerritsen, 2001, Partial amino acid sequence of purified von Willebrand factor–cleaving protease., Blood., 98, 1654, 10.1182/blood.V98.6.1654

Thông tin
Thông tin xuất bản

Blood

Tập 98 Số 6

1662-1666

Thông tin tác giả